Primary biliary cirrhosis( PBC) is an autoimmune cholestatic liver disease. In this review,the epidemiology of PBC,which varies in different countries and regions,is comprehensively introduced. Furthermore,the natural history of PBC before and after the wide application of ursodeoxycholic acid is analyzed. Also,recent advances in the study field of PBC in China are summarized. However,we still lag far behind Western countries concerning the diagnosis and treatment of PBC. Therefore,we believe further improving the knowledge of PBC among primary care physicians and PBC patients,setting up the multi- center PBC study group in China,and probing into the pathogenesis of this disease would pave the way for effective treatment of PBC patients in China.