Objective: To describe the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children.Method: Data from 11 pediatric patients with autoimmune GFAP astrocytopathywas retrospectively analyzed.Results: All of the patients showed encephalitis and meningoencephalitis or meningoencephalomyelitis with or without myelitis, include fever (45.4%), headaches (27.3%), dizziness (18.2%), drowsiness (18.2%) and mental disorders (18.2%). Cerebrospinal fluid (CSF) were detected in all patients. The white cell counts (WBC) (90.9%), lactic dehydrogenase levels (72.7%), protein level (36.4%), and adenosine deaminase activity (ADA) level (27.3%) were elevated, and the CSF glucose levels (72.7%) were slightly reduced. Nine patients (90%) were found to have brain abnormalities, of which five (50.0%) patients had abnormal symmetrical laminar patterns or line patterns hyperintensity lesions on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the basal ganglia, hypothalamus, subcortical white matter and periventricular white matter. The linear radial enhancement pattern of the cerebral white matter was only seen in two patients, the most common being abnormal enhancement of leptomeninges (50%). Five patients had longitudinally extensive spinal cord lesions.Conclusion: The findings of pediatric patients with autoimmune GFAP astrocytopathy is different from the previous reports.