Autoimmune hepatitis is a chronic inflammatory liver disease that responds well to prednisone alone or in combination with azathioprine. Combination therapy is preferred initially because of the lower rate of corticosteroid-induced adverse effects. Specific criteria for diagnosis include a wide range of biochemical, histologic, and immunologic features that define the disease. Autoimmune hepatitis is characterized by various autoantibodies, both traditional and nontraditional. Most of these autoantibodies are measured for diagnostic purposes and do not correlate with disease severity or activity. Sustained histologic remission is achievable in the majority of patients, although many patients require low-dose maintenance therapy. Drug therapy may be beneficial in patients with cirrhosis when considerable inflammation is noted on biopsy. Orthotopic liver transplantation should be considered for patients with decompensated cirrhosis due to autoimmune hepatitis or those with severe hepatitis in whom initial therapy is not successful.