Malignant rhabdoid tumor (MRT) is well known as a pediatric malignant tumor of the kidney. Only nine cases of primary MRT of the central nervous system (CNS) have been reported. We are reporting in detail the clinical course and treatment of a patient with primary MRT of the CNS. We cared for a 3-year-old girl with an MRT that extended from the internal auditory canal to the cerebellopontine angle. Despite three surgical attempts at resecting the tumor combined with whole craniospinal axis irradiation, as well as chemotherapy consisting of intravenous nimustine hydrochloride and intrathecal methotrexate injections, the patient died 13 months after her initial hospitalization. The origin of CNS-MRT development is still a question of pathologic debate. Like renal MRT, the prognosis of MRT of the CNS is very poor. The dissemination of MRT of the CNS occurred in most cases.