Congenital extrahepatic portocaval shunt associated with hepatic hyperplastic nodules in a patient with Dubin-Johnson syndrome.

We report a rare case of congenital extrahepatic portocaval shunt diagnosed during evaluation of hyperplastic nodules in the liver. Diagnostic imagings showed hypoplasia of the intrahepatic portal venous system and splanchnic portal venous return to the inferior vena cava through aberrant vessels. Altered hepatic blood flow dynamics due to this shunt may have been implicated in the etiology of the hepatic hyperplastic nodules.