Objective To analyze the causes of misdiagnosis of primary mediastinal ependymoma(PME).Methods The clinical data and imaging manifestations of a patient with PME treated in July 2023 were retrospectively analyzed.Re-sults In this patient,a painless mass was found in the mediastinum during physical examination,with no significant abnor-malities observed in laboratory tests.Chest CT showed posterior mediastinal cystic mass,considering the possibility of neuro-genic disease.Pathological smears after puncture biopsy of mediastinal cysts tended to be cysts of thymus origin.The patient underwent right thoracoscopic mediastinal cyst resection after admission,and the postoperative pathological diagnosis revealed PME.No recurrence or complications were observed during follow-up.Conclusion The occurrence of PME is extremely rare,which is more likely to be misdiagnosed as cysts of thymus or nerve origin.Clinical and imaging physicians should con-sider the possibility of this disease when they encounter lesions at this site,and the diagnosis should be combined with patho-logical and immunohistochemical examination.