Born in Turin, after joining Carrozzeria Pininfarina, he quickly became integral to the company, and during his career oversaw many of the designs (particularly Ferraris) for which the company is famous. In 1961 his family surname was changed from Farina to Pininfarina, to match that of the company, by decree of the Italian president.
In 1965 it was Sergio Pininfarina who personally persuaded Enzo Ferrari to adopt a "mid-engined" engine configuration, with the engine positioned behind the driver but ahead of the rear wheels, for a new line of road cars. The resulting Ferrari Dino Berlinette Speciale was presented at the Paris Motor Show in October, although it would be another two years before the cars were offered for sale.
After his father's death in 1966, Pininfarina became chairman of the company.