Glioblastoma is very rare in the pineal region. We report a case of glioblastoma in this region. This is the 18th case of primary glioblastoma in the pineal region and the second case that survived over two years according the literature. A 60-year-old man admitted with headache and ataxia that continued for the last 3 months. Physical examination was normal. Neurological examination revealed ataxia. There was no motor or sensory deficit. Computer tomography showed triventricular hydrocephalus and isodense rounded mass in the pineal region. Magnetic resonance images revealed a regular-edged heterogeneous contrast-enhanced tumor in pineal region. A ventriculoperitoneal shunt was inserted for hydrocephalus. After surgery, the ataxia and hydrocephalus were improved. Ten days later, serial stereotactic biopsies were performed. Histopathological specimens revealed glioblastoma. The patient was recommended to undergo radiotherapy and chemotherapy. The patient is still surviving without deficit two years after biopsy and shunt operation.