Primary biliary cirrhosis( PBC) is an autoimmune liver disease characterized by chronic and progressive cholestasis,which has a tendency to develop cirrhosis. The pathogenesis of PBC is complicated and still unclear. This paper reviews the latest research progress in immunological mechanisms and therapies for PBC in recent years,which focuses on the relationship between the development of PBC and autoantibodies,immune cells,and important cytokines. The review indicates that immunological factors play a key role in pathogenesis of PBC,and further research on immunological mechanisms might be quite important for the identification of pathogenesis and clinical treatment of PBC.