Objective To investigate the clinical manifestations, imaging characteristics, treatment and outcome of children with myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGADs), so as to improve the understanding of the disease. Methods Fourteen MOGAD children who were admitted to our department from Jan. 2018 to Dec. 2021 were retrospectively enrolled, and the general data, clinical manifestations, laboratory tests, imaging characteristics, as well as the treatment and outcome were collected and summarized for analysis. Results Of the 14 children with MOGAD, 6 were male and 8 were female, and the age of onset ranged from 2 to 12 years. Ten cases presented with acute disseminated encephalitis, 2 with neuromyelitis optica spectrum disorder, and 2 with optic neuritis; 3 cases combined with consciousness disorder, 5 with abnormal behaviors, and 6 with convulsive seizures. Magnetic resonance imaging suggested extensive involvement of abnormal signals, mainly involving the white matter, cerebellum, optic nerve and spinal cord. All children had positive peripheral blood myelin oligodendrocyte glycoprotein immunoglobulin G (titers of 1∶10 to 1∶1 000), and 3 cases were combined with positive cerebrospinal fluid anti-N-methyl-D-aspartate receptor antibodies. All patients were treated with high-dose methylprednisolone combined with gamma globulin in the acute phase, and 1 patient was treated with plasma exchange. At 0.5 to 2.0 years of follow-up, 9 cases had a monophasic course and recovered well; 5 cases had recurrent episodes and remitted after treatment with rituximab in 3 cases and cyclophosphamide in 2 cases. Conclusion The clinical manifestations of MOGAD in children are complex, and the imaging features are lack of specificity. In the acute phase, high-dose methylprednisolone combined with gamma globulin is effective, while some children have a multiphasic course of disease. Dynamic monitoring of myelin oligodendrocyte glycoprotein antibody level is helpful to early detection of patients with possible recurrence. Timely starting second-line drug treatment such as rituximab and cyclophosphamide can improve the prognosis.