Objective To explore the early diagnosis and standard treatment of autoimmune hepatitis (AIH). Methods  The process of clinical diagnosis and treatment in 2 children with AIH was retrospectively analyzed. The related literatures were reviewed. Results Two males were 6 years and 1 month old and 6 monthes and 18 days old, respectively. Both of them had the symptom of jaundice at onset, accompanied with the elevate aminotransferase and positive autoantibodies. One case showed elevated serum IgG and interface hepatitis in liver pathology, and all other causes were excluded. He had a good response to the treatment of hormone combined with azathioprine. The other case had a poor response to the treatment of single hormone. Conclusions Childhood AIH is rare in clinic. Its response to the therapy of glucocorticoid and immune inhibitors is good. So it should be diagnosised and treated early.