A case of accessory scrotum in a 1-year-old boy is reported because of its rarity. A boy presented with a tumor mass attached with scrotum-like skin on its tip in the right side of perineum between the scrotum and anus. Both testes had descended into the scrotum. There was no other urological anomaly. Histological findings of the tumor indicated perineal lipoma, and the scrotum-like portion accessory scrotum. An overview of sequences during the normal development of male external genitalia has been provided and the deranged mechanism resulting in this anomaly has been reviewed with hypothesis regarding etiology of accessory scrotum.