Objective To explore the causes and preventive measures of clinical misdiagnosis of brain glioma.Meth-ods The clinical data of 2 patients with misdiagnosed brain glioma admitted from May 2020 to October 2023 were retrospec-tively analyzed.Results A patient presented with dizziness and left limb weakness.Head MRI showed multiple abnormal signals,and the patient was misdiagnosed with cerebral infarction and central nervous system lymphoma,and finally diagnosed with cerebral glioma by biopsy.The patient declined surgery,and died after discharge.One patient presented with seizures of the left upper limb and loss of consciousness.Brain MRI showed abnormal signals in the right frontal cortex,and magnetic res-onance spectroscopy(MRS)showed elevated choline/N-acetylaspartate(Cho/NAA)in the focal area,which was misdiag-nosed as unilateral cerebral cortical encephalitis(UCCE)with positive myelin oligodendrocyte glycoprotein antibody,and fi-nally confirmed as brain glioma by histopathology.The patient was fine after surgery and did not have another seizure.Conclusion The clinical manifestations of brain glioma are complex,diverse,and lack of specificity.Therefore,for this kind of disease,clinicians need to repeatedly inquire about the medical history,combine clinical characteristics and other medical and technical examination,and perform pathological biopsy when necessary to make a correct diagnosis,to avoid misdiagnosis.