Objective To explore the diagnosis and treatment of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) in children. Methods The clinical data of an autoimmune GFAP-A in a child were retrospectively analyzed, and the related literature was reviewed. Results In a girl, aged 10 years and 2 months, it had an onset with fever. The child developed dyspnea and urine retention early in the course of the disease, followed by progressive disturbance of consciousness and convulsions. Her glial fibrillary acidic protein (GFAP) antibody in cerebrospinal fluid and serum were positive. Brain magnetic resonance imaging (MRI) showed bilateral thalamus and basal ganglia lesions. Plain and enhanced MRI scan of the spinal cord showed linear enhancement in the thoracic part of the spinal membrane and nerve roots. The child was finally diagnosed with GFAP-A, and his condition improved after treatment with gamma globulin and hormones. Conclusion GFAP-A begins with meningoencephalitis with or without myelitis. MRI showed meningeal enhancement, multiple hyperintensity lesions, and vascular-like radiologic enhancement. Antibody detection is helpful for diagnosis.