Demyelinating pseudotumor mainly attacks in teenagers and more commonly seen in female compared with male counterparts. Comprehensive understanding of medical history, clinical manifestations, imaging and pathological characteristics contribute to clinical diagnosis and management of demyelinating pseudotumor. Demyelinating pseudotumor in the central nervous system is mainly characterized as nerve fiber demyelination and inflammatory cell infiltration surrounding the minute vessels. However, the clinical and imaging characteristics of demyelinating pseudotumors mimic those of brain tumors, especially the brain glioma. It is likely to make a misdiagnosis of demyelinating pseudotumors. Herein, we reported one case of intracranial demyelinating pseudotumors and described the diagnosis and treatment of this patient in this study.