Although autoimmune liver diseases are common in Western countries, only sporadic cases have been reported in Korean children. Autoimmune hepatitis, primary biliary cirrhosis, autoimmune cholangiopathy and primary sclerosing cholangitis are major classes of liver diseases currently considered autoimmune. The overlap syndrome has features associated with both autoimmune hepatitis and other liver diseases. We treated a case of the overlap syndrome that included auoimmune hepatitis and cholangiopathy in a 12 year-old boy. Considering that these entities, autoimmune liver diseases or overlap syndromes, are potentially treatable diseases in children, a high index of suspicion is needed for the appropriate diagnosis.