Objective To study the treatment of multiple intraspinal and intracranial primitive neuroectodermal tumors(PNETs) by reporting a case and reviewing the literatures.MethodTo report a case of multiple intraspinal and intracranial PNETs in which 5 intraspinal surgery of tumors resection,2 times of routine radiotherapy of brain and spinal cord,ventricle-operitoneal shunt for hydrocephalus,and twice X-knife radiotherapy for tumor in 4~(th) ventricle and temperal lobe were performed.ResultsThe microscopic total removal of spinal tumors were achieved in all 5 intraspinal operations.The preoperative symptoms were relieved and there was no new postoperative neurological defect.The hydrocephalus disappeared after shunt operation.The 4~(th) ventricle tumor disappeared after X-knife radiotherapy.The recurrence of spinal tumor was observed 40 months after radiotherapy of brain and spinal cord.The patient died of renal failure after 78 months follow-up.ConclusionsMultiple intraspinal and intracranial PNETs is a really rare condition which may be with poor prognosis.Microscopic total resection of tumors combined with postoperative normalized radiotherapy is beneficial to prolong the living period of patient.