Autoimmune liver disease in children presents predominantly as autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC). These diagnoses must be considered in patients who have acute and chronic hepatitis, particularly when an extrahepatic autoimmune disorder is present. In AIH, the timely and sustained control of liver inflammation is critical to improve the short- and long-term outcomes. No effective treatment for PSC has been identified to date, but supportive care, careful attention to complications and associated nonhepatic diseases, and liver transplantation significantly improve the long-term outcome.