Introduction
Primary intracranial primitive neuroectodermal tumors (PNETs) are extremely rare malignancies that usually originate from the meninges and show varying degrees of neuroectodermal differentiation [1-4]. About 66 cases of these tumors have been reported so far. PNETs are poorly differentiated, highly malignant, and aggressive small round cell neoplasms. They had a high propensity for local recurrence and distant metastasis. Intracranial PNETs commonly affect children and adolescents [1, 2, 5]. It is noteworthy that the incidence of PNETs in adults is exceedingly rare. Only about ten cases of these tumors have been reported over the age of 33 years [1, 2]. Fusion of the EWSR1 gene with a member of the gene family is considered the primary cause of the PNETs [1, 4]. Due to the scarcity of cases of patients with PNETs, the clinical and imaging features, treatment and prognosis are not clear [1, 2].