Autoimmune hepatitis (AIH) is an important cause of chronic and progressive liver disease. Clinical presentation may vary from elevated liver enzymes to jaundice and, rarely, acute liver failure. A combination of elevated liver parameters, a positive autoantibody profile, and increased serum immunoglobulins with compatible liver histology are required for diagnosing AIH. Prompt recognition and treatment improve clinical outcomes. Disease control can be achieved by long-term corticosteroid therapy in tapering doses with azathioprine. Second-line agents are useful in case of intolerance to azathioprine. Patients with AIH should be followed up on a regular basis for disease flares and drug-related adverse reactions. Coexistence of primary biliary cirrhosis or primary sclerosing cholangitis defines overlap syndrome. AIH patients with acute liver failure presentation should be considered for liver transplantation.