Prune belly syndrome (PBS) is a triad of an abdominal wall muscular deficiency, cryptorchidism, and dilated urinary tract, and is often associated with other anomalies. Association of PBS with congenital pouch colon is extremely rare with only few handful of such reports in literature. We report here management of this rare associationin a male newborn with multiple genitourinary abnormalities. Keywords: Congenital pouch colon; Prune belly syndrome; High Anorectal malformation; Megaureter.