Budd-Chiari syndrome (BCS) is a venous outflow obstruction of the liver that has a dismal outcome if left untreated. Most cases of BCS in the Western world are caused by thrombosis of the hepatic veins, sometimes in combination with thrombosis of the inferior vena cava. Typical presentation consists of abdominal pain, hepatomegaly and ascites, although symptoms may vary significantly. Currently, a prothrombotic risk factor, either inherited or acquired, can be identified in the majority of patients. Moreover, in many patients with BCS a combination of risk factors is present. Myeloproliferative disorders are the most frequent underlying cause, occurring in approximately half of the patients. Recent discovery of the Janus Kinase 2 (JAK2) mutation has significantly contributed to the diagnosis of myeloproliferative disorders. Anticoagulation is indicated for all patients with BCS and additional therapy depends on the severity of symptoms and the extent of venous obstruction. A stepwise therapeutic approach is recommended, with increasing invasiveness and guided by the response to previous treatment. A transjugular intrahepatic portosystemic shunt (TIPS) is proving to be a good therapeutic option in patients with BCS, diminishing the need for surgical shunts. When all other therapy is unsuccessful or in patients with fulminant hepatic failure, a liver transplantation should be considered. Advances in diagnosis and treatment have dramatically improved the prognosis of patients with BCS. Still, many aspects of this complicated disorder remain to be clarified.