Hepatic vein thrombosis (Budd-Chiari Syndrome) is a rare disorder resulting from obstruction to the outflow of blood from the liver. The characteristic pathologic findings are intense congestion most pronounced around the terminal hepatic venules, cell necrosis, and a scant inflammatory reaction. Major clinical manifestations include hepatomegaly, right upper quadrant abdominal pain, and ascites. Disorders associated with hepatic vein thrombosis include those associated with a thrombotic tendency, such as polycythemia vera and paroxysmal nocturnal hemoglobinuria. Use of oral contraceptive agents probably increases tendency to develop hepatic vein thrombosis. Biochemical tests of the liver are of little value. The hepatic scan may be useful in suggesting the diagnosis with a marked decrease in uptake of isotope over affected areas of the liver. The diagnosis is confirmed by inferior vena caval and attempted hepatic venous catheterization. An associated thrombosis of the inferior vena cava is frequently found. Therapy in hepatic vein thrombosis is directed towards correction, in so far as possible, the underlying disorder, prevention of further extention of thrombosis, and management of ascites. Side-to-side portacaval or mesocaval shunt operation may convert the portal vein into an effective hepatic outflow vessel and reduce intrahepatic pressure and decrease injury. Mesoatrial shunts have proven useful in a few patients with hepatic vein thrombosis and either an inferior vena caval thrombosis or a marked pressure gradient from the abdomen to the thorax from ascites and an hypertrophied caudate lobe of the liver. For patients with extensive hepatocellular disease and portal hypertension, hepatic transplantation would seem to offer the best chance for survival.