Juvenile granulosa cell tumor is a rare benign neoplasm of the testicular stroma that accounts for 1-5% of all prepubertal testis tumors [Metcalfe PD, Farivar-Mohseni H, Farhat W, McLorie G, Khoury A, Bagli DJ. Pediatric testicular tumors: contemporary incidence and efficacy of testicular preserving surgery. J Urol 2003;170:2412-2416; Ross JH, Rybicki L, Kay R. Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the prepubertal testis tumor registry. J Urol 2002;168:1675-1679]. A prior case series retrospectively identified a cystic testis tumor on prenatal ultrasound images which was subsequently diagnosed as a juvenile granulosa cell tumor [Bryan DE, Cain MP, Casale AJ. Juvenile granulosa-theca cell (sex cord-stromal) tumor of the infant testis. J Urol 2003;169:1497-1498]. We report a case of a prenatally diagnosed testis tumor which was subsequently diagnosed as a juvenile granulosa cell tumor.