Rectal Atresia with Pouch Colon in a Male Child

Anorectal malformation is an important congenital condition characterized by an absent anal opening. Among its varied presentations, rectal atresia (RA) is a type in which the rectum terminates blindly without a fistulous connection. However, the anus is present and is patent for about 1–2 cm. Congenital pouch colon (CPC) is a rare condition associated with anorectal agenesis, characterized by an abnormal colon dilation. This abnormal pouch terminates in the urethral tract through a fistula. The association of RA with CPC is extremely uncommon. We operated on a male neonate having RA associated with a CPC. The diagnosis of pouch colon was suspected based on an X-ray, and it was confirmed intraoperatively. Being an extremely uncommon clinical entity, it is presented with a review of the relevant literature.