A congenital neoplasm arising in the central nervous system is rarely encountered, and the majority of case reports that have dealt with intracranial tumors have been divided almost equally between teratomas and various gliomas. We experienced a rare case of congenital glioblastoma multiforme encountered in a three day-old male infant who presented with hydrocephalus since birth. Post-mortem examination revealed that the tumor seemed to have originated from the right thalamic region extending centrifugally to the cerebral cortex and through the brain-stem down to the cerebellum.