Perineal accessory scrotum with a lipomatous hamartoma in an adult male

Accessory scrotum is a rare congenital anomaly that is often associated with perineal tumor or other developmental abnormalities. Because most cases are diagnosed and treated in early childhood, little is known about the natural biologic course of this entity and associated lesions through time. We present a case of accessory scrotum associated with lipomatous hamartoma in a 46-year-old man who was born with a perineal mass. We evaluate the clinicopathologic features and discuss the pathogenesis with a review of the literature.