Seyung Kim, Jae Rock Oh, Jin Bum Kim, Seong Ho Lee, Sang Kon Lee, Im Kyung Hwang, Myung Sun Hong, Young Hee Choi From the Departments of Urology, Radiology, Pathology, School of Medicine, Hallym University, Seoul, Korea The presence of accessory scrotum is a condition characterized by additional scrotal tissue with a normally developed scrotum. The condition is extremely rare and can be associated with a perineal lipoma. Herein, a case of accessory scrotum associated with a perineal lipoma is reported in a 4-day-old boy. He presented with a perineal mass and the diagnosis was confirmed by surgical excision and subsequent histological analysis. This is the first Korean report of an accessory scrotum associated with perineal lipoma. (Korean J Urol 2007;48:1096-1098)