• The diagnosis of autoimmune hepatitis is based on specific immunological, histological and biochemical characteristics after exclusion of other causes of liver disease including viral hepatitis. • Hepatitis E virus infection should be considered in the differential diagnosis of autoimmune hepatitis. • Prednisone, prednisolone, or budesonide are first line treatment options for autoimmune hepatitis with or without addition of azathioprine. Treatment is indefinite in the majority of patients. • Detection of antimitochondrial antibodies is a hallmark of for the diagnosis of primary biliary cirrhosis. • Ursodeoxycholic acid is indicated for the treatment of primary biliary cirrhosis leading to improved clinical long-term outcome. • Overlap syndromes can occur between the different cholestatic liver diseases and AIH and may show dynamic patterns over time with changes in the dominant disease. Individualized treatment approaches are required.