A boy with perineal accessory scrotum but without any other congenital anomaly is being described. He presented with a rugose skin tag on a midline perineal mound and the diagnosis could be confirmed by the histological findings of subcutaneous smooth muscles. Other reports of perineal scrota have been reviewed and a simple classification has been proposed for such cases, based on the appearance of the accessory scrotum and the associated anomalies.