Abstract: Objective: To improve the the doctors' recognition in Budd-Chiari syndrome and reduce the misdiagnosis. Methods: The clinical data of 56 cases of Budd-Chiari syndrome from 2007 to 2010 were analyzed retrospectively. Results: This study consisted of 33 males and 23 females aged from 20 to 70 with a disease course of 6 months to 25 years. The clinical symptoms primarily included abdominal distension, double leg edema and liver enlargement; the auxiliary examination methods mainly included color Doppler ultrasound and inferior vena cava imaging. Twenty-five cases were treated by PTA and STENT; 30 cases had been misdiagnosed as liver cirrhosis. Conclusions: The clinical features of Budd-Chiari syndrome should be noticed to reduce misdiagnosis. Early diagnose and active treatment may effectively improve the prognosis of the patients.