Budd-Chiari syndrome is the generic term for different forms of hepatic venous outflow obstruction resulting in a clinical picture of portal hypertension and hepatomegaly. Three levels of venous outflow obstruction may be recognized, affecting respectively the small intrahepatic (IVC). Each level of obstruction is related to a different aetiology. Clinical manifestations range from mild symptoms to acute or chronic end-stage liver disease. Treatment is surgical in the great majority of patients. Occlusion of the IVC may be treated by removal of the caval obstruction in selected patients. Hepatic outflow obstruction may be circumvented by different forms of shunting from the portal or upper mesenteric vein to the IVC or right atrium, depending on the level of obstruction and the difference in venous pressure. For the rare patient presenting with acute or chronic end-stage liver failure, hepatic transplantation may be a life-saving procedure.