Disturbances in the immune network are believed to be the key step in the pathogenesis of autoimmune liver diseases. Contributing factors to these disarrangements may include aberrant expression of major histocompatibility complex molecules, as well as the expression of primarily intracellularly located antigens on cell surfaces, making them accessible to the immune system. A distinct immunogenetic background of autoimmune liver diseases is now well established, but shows, however, geographic differences. The detailed characterization of the targets of both humoral and cellular immune reactions provides valuable tools for the identification and classification of autoimmune liver diseases and allows new insights into its pathogenesis. Autoimmune liver diseases can be triggered by hepatotropic viruses, especially chronic hepatitis C, which can be accompanied by autoantibodies. Thus, the distinction between idiopathic autoimmune chronic active hepatitis and chronic hepatitis C is very important for decisions on treatment. Therapy of idiopathic autoimmune chronic active hepatitis relies on immunosuppressive agents, and orthotopic liver transplantation offers a favorable prognosis to those who do not sufficiently respond.