Autoimmune hepatitis (AIH) is a rare chronic inflammatory liver disease that affects all age groups and, without adequate treatment, can lead to liver failure. Early diagnosis and treatment are crucial for a favorable prognosis of AIH. However, the diagnosis may be complicated by the heterogeneous spectrum of clinical, laboratory, and histological manifestations. Increased transaminases and immunoglobulin G levels, positive autoantibodies, characteristic morphological changes in liver histology, and finally, good response to immunosuppressive treatment are among the most important diagnostic criteria and should be evaluated if suspected. The goal of therapy should be to achieve complete clinical, biochemical and histologic response. This can be achieved by immunosuppressive therapy with corticosteroids and azathioprine in most of the patients. However, while many patients initially respond well to standard immunosuppressive therapy, side effects or inadequate treatment response continue to be a challenge in the treatment of AIH. This underscores the need for prospective studies for effective alternative treatment. © 2018, Springer Medizin. All rights reserved.