Agenesis of the right lobe of the liver is an extremely rare anomaly of the liver, and few cases are reported in the literature. Most of the patients with this anomaly are accompanied by additional anormalies such as retrohepatic or suprahepatic gallbladder and other biliary tract diseases, including cholelithiasis, carcinoma of the gallbladder and portal hypertension. The diagnosis of this rare anatomical variant was established by ultrasonography and computed tomography. The radiological findings, clinical presentation, and differential diagnosis are reviewed.(Korean J Hepatol 2000;6:124－128)
