Being one of the first conditions in which specific autoantibodies were recognized, PBC is regarded as a "model autoimmune disease." Both environmental factors and inherited genetic predisposition appear to contribute to its pathogenesis [7] . Although there has been tremendous progress in unraveling potential pathophysiologic factors in PBC over the past years [7] , the actual impact of each of the identified genetic and environmental associations is still controversial. It is the aim of this article to review the current knowledge of major pathologic features in PBC and to try to combine these findings to an overall picture of the pathogenesis of PBC.