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Budd-Chiari Syndrome (BCS) is a rare cause of portal hypertension. Geographical variations occur, particularly in the clinical presentation, where there are distinct differences between West and South Asia. Idiopathic forms are common in south Asia, while hypercoagulable disorders are common causes of BCS in the West. The site of thrombosis is also different, with patients from South Asia presenting with combined obstruction of the hepatic veins and the inferior vena cava in contrast to isolated obstruction of the hepatic veins, common in the West. Ultrasound-Doppler studies confirm the diagnosis in the majority. Early radiological interventions, including transjugular intrahepatic portosystemic shunt, can cure the majority of cases with idiopathic forms. Prothrombotic forms are treated with long-term anticoagulants. Surgery is reserved to a selected few with long segment obstruction. Liver transplantation is indicated in patients with worsening clinical functional status not responding to medical and/or interventional management.