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儿童自身免疫性肝病不是很常见，除了自身免疫性肝炎，还包括自身免疫性硬化性胆管炎、巨细胞肝炎伴自身免疫性溶血、肝移植后新发自身免疫性肝炎以及2个获得性自身免疫性肝病：新生儿狼疮、妊娠同种免疫性肝病(又名新生儿血色病)。涉及本年龄阶段特有的免疫系统和全身的发育特点，决定着儿童的自身免疫性肝病的种类、临床特征、预后转归可能不同于成人。现就临床越来越多发的儿童自身免疫性肝炎发病机制、抗体、病理改变以及诊治进展展开论述。“,”Autoimmune liver disease is not common in children. In addition to autoimmune hepatitis, it also includes autoimmune sclerosing cholangitis, Giant cell hepatitis with autoimmune hemolytic anemia, and de novo autoimmune hepatitis after liver transplantation as well as two acquired autoimmune liver diseases: neonatal lupus and Gestational alloimmune liver disease (alternate name neonatal hemochromatosis). The age-specific systemic developmental characteristics and immune system association determine the type of autoimmune liver disease in children, and its clinical manifestations and prognostic transition may vary from adults. Here, we discuss the rising clinical incidence of autoimmune hepatitis in children.