Abstract: This paper reviewed the research progress of Budd-Chiari syndrome, a portal hypertension caused by hepatic venous occlusion.Budd and Chiari reported their findings of the occlusive status of the hepatic veins in 1845 and 1899 respectively.Since then etiologic and epidemiologic investigations on the disease were carried out.Renomination of Budd-Chiari syndrome to the hepatocaval occlusive syndrome is perhaps much more appropriate because the occlusive lesions were mainly located in the inferior vena cava in China, India, and South Africa.According to the classification of lesions, different treatments were developed.In the acute stage, the fibrolysis therapy is indicated.In the chronic stage, it includes various PTA, indirect decompression, peri-gastroesophageal devascularization, promoting collateral procedures, direct decompression, including cavo-or meso-even capitus medusa to right atrium or internal jugular vein shunt and the radical resection is a means for radical cure.However, major surgery and liver transplantation is the last option and the indication must be strict.Further research should focus on the etiology of the membranous obstruction of the inferior vena cava, early diagnosis and early intervention and PTA or surgery in treating the more complex lesions.