Budd-Chiari syndrome (BCS) is defined as the obstruction of the outflow of the hepatic venous system which may occur at any level between the hepatic venules and the inferior vena cava (IVC)—right atrium junction, irrespective of the cause of obstruction. Outflow obstruction caused by cardiac disorders and veno-occlusive disease of the liver is not included in this definition [1]. As far as pathophysiology is concerned, obstruction of the venous outflow from the liver leads to congestion and stasis, causing elevation of pressure within the sinusoids and a resultant hypoxic damage to hepatocytes and the sinusoidal endothelium. Hepatocyte necrosis then ensues along with gradually increasing centrilobular fibrosis, nodular regenerative hyperplasia and finally cirrhosis [2, 3]. However, liver function can be preserved if the pressure within the sinusoids is brought down by re-establishing the outflow system, forming a portosystemic shunt or by the formation of venous collaterals. Therefore, it is important to understand the underlying causative factors, clinical features and baseline liver function for choosing the best management plan for patients which may vary from conservative therapy to endovascular management to liver transplantation.