Objective To investigate the clinical features and treatment outcome of children with autoimmune hepatitis ( AIH) . Methods A retrospective analysis was performed for the clinical data, liver pathology, treatment outcome, and follow-up data of 10 children with AIH who were treated in Beijing YouAn Hospital, Capital Medical University, from December 2008 to December 2017. Results The children were aged 3-16 years ( median 10 years) , and girls accounted for 70%. Of all patients, 5 had type 1 AIH, and 5 had type 2 AIH; 3 ( 30%) had an acute onset, 2 ( 20%) had a subacute onset, and 5 ( 50%) had a chronic onset. Elevation of aminotransferases was found in 8 children ( 80%) , and elevations of bilirubin, gamma-glutamyl transpeptidase, and immunoglobulin G were found in 7 children ( 70%) , 7 children ( 70%) , and 6 children ( 60%) , respectively. Among the 7 children who underwent liver biopsy, 3 had grade ≥3 liver inflammation, 4 had stage ≥3 liver fibrosis, 5 had interface hepatitis, 5 had plasma cell infiltration, 4 had rosette-like annulation of hepatocytes, and 7 had lymphocyte infiltration. One child died, and one was lost to follow-up; among the other 8 children, 6 had good response, 1 experienced recurrence, and one had poor response. 4 patients with type 2 AIH were treated with glucocorticoids combined with azathioprine, the disease was effectively controlled. Conclusion Children with AIH have diverse clinical manifestations, and some children have serious conditions. Most patients with type 2 AIH need the treatment of glucocorticoids combined with azathioprine.