A case is presented of a patient with bilateral gonadoblastoma characterized by primary amenorrhea and intense, generalized hirsutism. The physical examination showed somatic stigmas compatible with gonadal dysgenesis. Pneumopelvigraphy showed a uterus and hypoplastic gonads; no microcalcification was seen. Negative sex chromatin in buccal mucosal cells and a 46, XXr/46,XXp-q-45,X karyotypic constitution was found.