Central primitive neuroectodermal tumors are relatively rare among primitive neuroectodermal tumors. First reported by Hart in 1973, primitive neuroectodermal tumors are relatively rare and highly malignant neurological tumors, which are more primitive tumors derived from the neural crest, arising mainly from the primitive neuroepithelium, and have multidirectional differentiation potential. With aggressive growth and extensive cerebrospinal fluid dissemination, the prognosis is extremely poor, and some of them still require pathologic diagnosis to confirm the diagnosis. Histomorphology belongs to malignant small round cell tumors, central and peripheral, peripheral is more common, more articles reported, central primitive neuroectodermal tumors are relatively rare, and supratentorial primitive neuroectodermal tumors are rare, only account for about 0.1% of the whole brain tumors, and are mostly seen in children hours, adults are very rare. Symptoms of central primitive neuroectodermal tumors: headache, vomiting, intracranial hypertension with limb weakness and convulsions, with gait instability, simple headache. Treatment of central primitive neuroectodermal tumor: generally there is no special treatment, usually surgery, and then supplemented with radiotherapy and chemotherapy and other comprehensive treatment measures after surgery.