Juvenile granulosa cell tumor occurred in a newborn. The tumor presented with testicular torsion, and no malformations were observed. The karyotype was normal. The occurrence of initial tumoral lesions in the seminiferous tubules located in the vicinity of the tumor suggests that the tumor originated from immature Sertoli's cells. To our knowledge, this is the tenth case reported in a newborn and the second associated with testicular torsion.