Agenesis of gallbladder with choledochal cyst--an unusual combination.

A case of agenesis of gallbladder associated with Type IVa congenital choledochal cyst is reported. This combination is very rare and a review of literature revealed only three such cases. A brief discussion of gall bladder agenesis with particular reference to associated anomalies is presented. Difficulty in the management of Type IVa choledochal cyst where the extrahepatic and intrahepatic cysts are continuous at the porta, is highlighted.