Mears was born in Wichita, Kansas, and raised in Bakersfield, California. He began his racing career in off-road racing. In 1976, a representative of Bill Simpson's helmet company recommended him, and Simpson gave Mears a ride at the USAC Champ Car's California 500 on an old Eagle-Offenhauser; he finished eighth. Simpson then sold the car to Art Sugai on condition that Mears would continue driving it. In mid-1977 Mears switched to Theodore Racing.
Mears's speed attracted the attention of Roger Penske. In 1978, with Penske Racing already running Mario Andretti and Tom Sneva, Mears was offered a part-time ride in nine of the 18 championship races, filling in when Andretti was competing in Formula One with Lotus. The arrangement included a seat at the Indianapolis 500.
In his rookie appearance at Indianapolis, Mears qualified on the front row and was the first rookie to qualify at over 200 mph. When the race started, he discovered his helmet was not fastened correctly and had to pit to have it secured; he retired at lap 104 with a blown engine. He shared Rookie of the Year honours with Larry Rice. Two weeks later at the Rex Mays Classic at Milwaukee he won his first race; further wins followed at Atlanta and at Brands Hatch on a road course.
Following Tom Sneva's departure from Penske, Mears was elevated to full-time status for 1979, the year the national championship sanction changed from USAC to CART. At Indianapolis he won his first 500, taking advantage when Bobby Unser fell out of contention with mechanical trouble. Three wins and four second places in the eleven CART-eligible races secured Mears his first championship; his worst finish that season was seventh in Trenton's second heat.
The 1981 and 1982 seasons brought two further championships. Despite suffering facial burns in a pit fire during the 1981 Indianapolis 500, Mears accumulated ten race victories across the two years. At the 1982 Indianapolis 500 he came within 0.16 of a second of Gordon Johncock, a margin that stood as the closest finish in Indy 500 history for ten years. In the closing laps, a full fuel load during Mears's final pit stop left him over eleven seconds behind Johncock; he closed the gap when Johncock suffered handling problems but could not complete the overtake. The race was also shadowed by controversy after drivers including Johncock, Johnny Rutherford, and Bobby Unser accused Mears of causing a pace-lap crash involving teammate Kevin Cogan.
For 1983 the Penske team adopted Pennzoil sponsorship and its yellow paint scheme, with Al Unser taking the championship. Mears switched to the March chassis for the 1984 Indianapolis 500 after the Penske chassis proved unsuccessful in the opening two races. He won his second Indy 500 that May, but later in the year a crash at Sanair Super Speedway inflicted severe leg injuries — particularly to his right foot — that hampered him throughout the rest of his career. The March chassis, like most contemporary open-wheel cars, offered little protection for the driver's legs and feet.
Despite the injuries, Mears won the 1985 Pocono 500 as a comeback milestone. In 1986 he won the pole at Indianapolis and finished third in the race. He also won the 1987 Pocono 500.
In 1988, the Penske team introduced the new PC-17 chassis with a Chevrolet racing engine. Mears used it to win his third Indianapolis 500. In 1989 he set a record fifth pole position at Indy but retired from the race with mechanical problems; Emerson Fittipaldi won both the 500 and the championship, defeating Mears at the final race at Laguna Seca Raceway, despite Mears winning that race. That victory at Laguna Seca established Mears as the most successful IndyCar racer of the 1980s.
Fittipaldi joined the Penske team for 1990, and Marlboro replaced Pennzoil as team sponsor. The 1990 season was dominated by Al Unser Jr., who scored six wins.
In 1991, during a practice session, Mears struck the wall at Indianapolis for the first time in his career. The following day he climbed into his backup car and claimed his record sixth career pole. In the closing stages of the 500, with twenty laps remaining, Michael Andretti held a fifteen-second lead. A yellow flag erased that margin, and Mears gained the lead when Andretti pitted for fuel. Andretti passed Mears around the outside entering turn one; a lap later Mears regained the lead using the same move, then extended the gap to win his fourth Indy 500, becoming the third driver to reach that total. In August 1991 he won his final career race at the Michigan 500.
In 1980, amid uncertainty over the CART–USAC split, Mears accepted a Formula One test with Brabham offered by team boss Bernie Ecclestone. He tested at Paul Ricard and at Riverside. After adjusting to the Brabham BT49, he posted lap times within half a second of then-Brabham driver Nelson Piquet at the first test, and faster than Piquet's times at the second. Piquet endorsed signing Mears as a second driver, and Ecclestone offered a contract. Mears declined, citing his preference for oval racing and uncertainty about relocating to Europe.
At the 1992 Indianapolis 500, Mears broke a wrist in a practice crash and subsequently crashed out of the race itself — the first time in his career he had failed to finish at Indy. He contested only four more races in 1992 and announced his retirement at the Penske team's Christmas party; only Penske and Mears's wife Chris were aware of his plans in advance. He was 41 years old.
As of 2023, Mears continues to work as a consultant and spotter for Penske Racing, mentoring drivers including Helio Castroneves, Will Power, and Scott McLaughlin. He was inducted into the International Motorsports Hall of Fame in 1997, the Indianapolis Motor Speedway Hall of Fame in 1998, the Motorsports Hall of Fame of America in 1998, and the Team Penske Hall of Fame on 25 May 2017.
Mears is the brother of Roger Mears, father of off-road and open-wheel racer Clint Mears, and uncle of part-time NASCAR Cup Series driver Casey Mears. His first marriage, to Dina, ended in divorce in 1983; he married Chris Bowen in 1986.
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