Budd-Chiari syndrome is a post-hepatic portal hypertension due to occlusion of the hepatic veins and/or the suprahepatic inferior vena cava (IVC), often accompanied with IVC syndrome. The causes of BCS are variable, and its clinical manifestations are also diverse. This paper reviews the current literature concerning the clinical manifestations, diagnosis and management of BCS, and endovascular and surgical treatment of this disease are highlighted.