The management of autoimmune liver disease can be very challenging. The presentation and natural history of these disorders is highly variable and can be asymptomatic, acute or chronic. Diagnosis requires the skilled interpretation of serological markers and histological changes and sometimes of biliary tract imaging. The major treatment options are immunosuppressive therapy (steroids and azathioprine) in autoimmune hepatitis (AIH) and ursodeoxycholic acid in cholestatic conditions although other treatments continue to be developed and tested. The complications of these diseases, in particular hepatocellular carcinoma and, in PSC, cholangiocarcinoma, remain difficult to monitor or prevent. Liver transplantation remains the only therapeutic option for end stage of liver disease and determining the optimum time for a patient to undergo this procedure requires a sophisticated judgment of the risks and benefits of the procedure as they pertain to an individual patient.