Autoimmune hepatitis is a self-perpetuating hepatocellular inflammation. The diagnosis is established by a number of diagnostic criteria, defined by the International Autoimmune Hepatitis Group, and the exclusion of other causes of chronic hepatitis. There are two fundamental goals in therapy: induction of remission and maintenance of remission. The standard initial treatment is prednisone monotherapy or combination therapy with prednisone and azathioprine, which induce a clinical, biochemical and histologic remission in 65-87% of patients within 3 years. Other typical treatment endpoints in autoimmune hepatitis are an incomplete response, treatment failure and intolerance of the administrated drugs. If the treatment results are unsatisfactory, liver transplantation and alternative drugs such as Cyclosporin A, tacrolimus, cyclophosphamide, mycophenolate mofetil, budesonide, ursodeoxycholic acid should be considered; however, efficacy in clinical trials has not been shown. Future investigations must focus on the clarification of pathogenic mechanisms, characterization of target autoantigens, identification of host susceptibility factors, and assessment of alternative treatment strategies.