Background: Primary intracranial Ewing sarcoma/peripheral primitive neuroectodermal tumor (EW S/pPNET) is exceedingly rare; and easy to misdiagnose.Case presentation: We report a case of a 23-year-old male who presented with headache and vomiting. The preoperative imaging examination of the brain revealed an irregular mass in the left parietal lobe, which was misdiagnosed as a meningioma, but the surgical specimen was diagnosed as primary intracranial EWS/pPNET. The patient underwent a total tumor resection, followed by adjuvant chemotherapy and radiotherapy. No recurrence or distant metastasis was found 18 months post-surgery. In addition, the clinical manifestations and treatment prognosis of primary intracranial EWS/pPNET were explored through a literature review.Conclusions: When the imaging features of young patients’ lesions are solid, aggressive, and unevenly enhanced masses, physicians should be aware of the possibility of primary intracranial EWS/pPNET, and if possible, gross total resection (GTR) and intensive chemotherapy and radiotherapy are recommended.