Neonatal central nervous system (CNS) tumors are an uncommon and histologically heterogeneous group of neoplasms with different clinical and biological features from those arising in childhood. We report 9 cases in which a diagnosis of CNS tumor was confirmed by biopsy or autopsy during the years 1982-1997 in the Vall d'Hebrón Children's Hospital, Barcelona. Two cases were fetal tumors detected by fetal sonography, 3 patients were symptomatic in the first days after birth and 4 patients presented initial clinical signs in the first weeks or months of life. Eight lesions were supratentorial and 1 was located in the spinal cord. According to histologic types, there were 2 glioneuronal tumors, 1 anaplastic astrocytoma, 1 choroid plexus carcinoma, 1 immature teratoma, 1 craniopharyngioma, 1 hemangioblastoma, 1 astroblastoma and 1 hemangioendothelioma. Extensive review of the literature indicates that our cases of hemangioblastoma, astroblastoma and hemangioendothelioma are exceptional and one more of the very rare and isolated previously published cases.