Budd- Chiari syndrome( BCS) is characterized by the hepatic outflow obstruction from the small hepatic vein to the superior hepatic inferior vena cava. Portal vein thrombosis( PVT) refers to the development of thrombosis in the main portal vein,with or without thrombosis in the superior mesenteric or splenic vein. A large number of studies have shown that prothrombin( factor II,F2) G20210 A mutation is related to BCS and PVT. F2 and its G20210 A mutation are introduced,the effect of F2 G20210 A mutation on thrombosis is reviewed,and the prevalence of F2 G20210 A mutation in China is analyzed. An attempt is made to focus on the association of F2 G20210 A mutation with PVT and BCS. It is believed that F2 G20210 A mutation to some extent increases the risk for PVT and BCS. However,the prevalence of F2 G20210 A mutation is extremely low in the Chinese population. Thus,this mutation may not be regarded as the cause of PVT and BCS in China.