Although various neoplasms may develop in the ventricular system, glioblastomas are rare. An 80-year-old woman visited our hospital with intractable headache related to a right ventricular large mass, which exhibited heterogeneous enhancement involving the body, trigone, and occipital horn of the right lateral ventricle on MRI. The mass was diagnosed as glioblastoma multiforme on surgical pathology. Herein, the authors present a case and review the existing literature with regarding to incidence, pathophysiology, prognostic factors, imaging and pathologic findings of intraventricular glioblastoma multiforme.