Aneurysmal malformation of the extrahepatic portal vein

Malformations of the extrahepatic portal vein are quite rare. A 6-cm aneurysmal malformation of the extrahepatic portal vein, thought to be congenital in origin, is described. An English language literature review yielded only six similar cases, each of which was associated with portal hypertension, primary hepatocellular disease, or both. The patient reported herein had cholelithiasis with chronic cholecystitis, but no primary liver disease or vascular disease. We believe this is the first report of such an anomaly.