Congenital pouch colon (CPC), a condition associated with anorectal agenesis has been reported mainly from the Indian subcontinent though there have been few case reports from other areas. The pouch differs from a normal colon structurally, histologically and functionally. The management involves a diversion colostomy at birth with or without the excision of pouch followed by pull through. This review article attempts to compile all the available literature on pouch colon along with the authors′ experience and discuss the relevant issues for proper diagnosis and management. A changing trend has been seen in the most common type of CPC seen over the years from complete congenital pouch colon that accounted for more than 70% of cases earlier to incomplete pouch colon that is more commonly seen now.