A case of the rare primary intracerebral neuroblastoma is reported. The patient, a 13-year-old girl, was subjected to craniotomy because of a right frontal tumor. The removed tumor tissue was composed of immature, highly cellular areas with numerous mitotic figures, adjacent to fields with Homer-Wright rosettes, and areas consisting of neuroblasts and mature neurons. There was no evidence of a primary tumor elsewhere in the body and 25 months after the operation and postoperative irradiation, the patient is without any signs of recurrence. Determination of catecholamines and their metabolites in the urine and cerebrospinal fluid gave normal results. Dense-corded vesicles could not be detected in the tumor cells by electron microscopy. A review of previously reported cases of primary intracerebral neuroblastomas is included. The cell of origin and the possibility of transition of a differentiating intracerebral neuroblastoma to ganglioneuroma are discussed.