Abstract: BuddChiari syndrome is rare disorder by hepatic venous outflow obstraction and resulting portal hypertension and（or）hypertension of infeior vena cava.Etiological factors include hypercoagulable conditions，myeloproliferative desenses and enviroment conditions.The diagnosis is establisbed by demonstrating obstruction of hepatic venous outflow by ultrasound，computed tomography or magnetic resonance.The main goals of treatment are to ameliorate hepatic congestion and prevent further thrombosis.The selective use of anticoagulation，revascularization，transjugular intrahepatic portosystemic shunt has resulted in a significant increase.Orthotopic liver transplant should be used as a rescue therapy.The epidemiology，etiology and pathogenesis，diagnosis，mangement and longterm followup of patiens with budd-Chiari syndrome is reviewed.

Key words: BuddChiari syndrome, balloon dilatation, transjugular intrahepatic portosystemic shunt