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 rare congenital malformation that is almost nonexistent outside the Indian subcontinent. However, this anomaly was initially recognized in England during the beginning of the twentieth century. The embryogenesis of CPC is not known, and the etiology is still quite elusive. The widespread use of and direct contact with pesticides in agriculture-based communities have been regarded as the possible factor in triggering of events that lead to CPC. CPC is recognized by certain pathological characteristics that are solely found to be associated with this congenital malformation. Histological studies of resected pouch colon demonstrate extreme variations in CPC.