Congenital pouch colon (CPC) is a malformation of the colon in which the entire large bowel or segments of varying lengths of the large bowel exhibit enormous dilatations in the form of a pouch and communicate with a distal fistula to the urogenital system. CPC is a condition that comprises a high form of anorectal malformation associated with large variations in the size of the dilatations of the affected colonic segment. The scarce reporting of this condition in the 1980s precluded its inclusion in the Wingspread classification of anorectal malformations; however, with the increasing number of reports and detailed investigations of the condition, CPC has been recognized as a rare form of anorectal malformation and has been included in the Krickenbeck classification.1