OBJECTIVE: To review an update of autoimmune hepatitis in children, based on classification, diagnostic and therapeutic criteria. METHODS: Articles on the theme were selected and updated, and the author's experience at the Liver Unit, Children's Hospital, School of Medicine, Universidade de São Paulo, was considered as well. RESULTS AND CONCLUSIONS: Autoimmune hepatitis has clinical features suggestive of acute viral hepatitis; however, other clinical features, laboratory tests, endoscopic and histological findings pointed to chronic liver disease, and the majority of the cases evolved to hepatic cirrhosis. The treatment with corticosteroids and/or azathioprine altered the natural history of autoimmune hepatitis, and the survival was 80% in 10 years, although hepatic cirrhosis was present at the beginning, without hepatic decompensation.