An anomaly of the extrahepatic biliary system is reported in which the
common hepatic duct was found to enter the gallbladder, whereas the c
ystic duct drained the whole biliary system into the duodenum. Review
of the literature revealed only eight previously reported similar case
s. To ascertain such anatomy, a choledochal cyst and the Mirizzi syndr
ome must be excluded. In the past, the rarity of the configuration des
cribed herein led to transection of the common hepatic duct during cho
lecystectomy in most cases. The concomitant presence of other abdomina
l anomalies, as in our case, or severe inflammation in the porta hepat
is should prompt suspicion of biliary anomalous anatomy. In that case,
dissection of the gall bladder from the fundus downward will allow ti
mely discovery of such an anomaly. Maintenance of continuity between t
he common hepatic duct and cystic-common biliary duct by preserving pa
rt of the gallbladder permits easy repair on a T tube.