Accessory scrotum and congenital perineal lipoma are unusual congenital anomalies. Congenital pulmonary airway malformation (CPAM) is a rare congenital disorder of the lung. There are five types of CPAM, each with a different clinical presentation and prognosis. The present report involves a case of type 2 CPAM associated with accessory scrotum and congenital perianal lipoma.