We report a father and son with striopallidodentate calcifications. Metabolic studies excluded calcium/phosphorus metabolism disturbances and no specific etiology was found. The structure of the calcified areas differed, on magnetic resonance imaging, depending on location and, probably, age. There are nine families with similar clinical and radiologic backgrounds and no evident etiology in the literature. Transmission is most often autosomal dominant, and in contrast with physiologic senescent basal ganglia calcification, the prognosis appears to be poor.