Background: Autoimmune hepatitis (AIH) is considered rare, and it is even rarer in the pediatric population. AIH in the pediatric population is categorized into type 1 and 2, which are differentiated by their autoantibody profiles. Case report: 5 years old Saudi boy presented with history of jaundice, fever and dark urine for 5 days with previous similar history at the age of 3 years. Examination revealed that he was conscious, looks pale and jaundiced but not in respiratory distress and well hydrated. Abdominal examination showed hepatosplenomegaly. Anti-nuclear antibodies (ANA), antismooth muscle antibodies and anti-neutrophilantibodies were positive whereas anti platelet antibodies and anti-mitochondrial antibodies were negative. Hepatitis serology was negative. Magnetic resonant cholangiopancreatogram (MRCP) showed diffuse periportal edema, likely related to liver cirrhosis. Liver biopsy was suggesting end stage biliary cirrhosis. Diagnosis was end stage cirrhosis with biliary features secondary to autoimmune process. The patient was treated with prednisolone, ursodeoxycholic acid, Vitamin K, omeprazol and iron. He was discharged and listed on the liver transplant clinic. Conclusion: This case report highlights and alerts physicians that AIH should always be considered in paediatric patients presenting with chronic liver disease.