Autoimmune hepatitis is a chronic liver disease characterized by a necroinflammatory activity of the portal tract resulting from an immune response against hepatocytes. It is relatively rare and its cause is unknown. There are three types (I, II and III) associated with the presence of some autoantibodies which are not always specific. The diagnosis is based on clinical, biochemical, immunological and histological features. The preferred treatment is a combination regimen of predniso(lo)ne and azathioprine (budezonide and aziathioprine in when cirrhosis is absent). The corticosteroid must be slowly tapered down keeping the immunosuppressive agent as maintenance therapy. Drug withdrawal should only be considered whether serum transaminases and γ-globulins level are normal and after a normal or near normal liver biopsy.