A third ventricle tumor, in addition to a recurrent cerebellar hemangioblastoma, was found in a 47-year-old woman on follow-up magnetic resonance imaging (MRI) 5 years after operation of the cerebellar tumor. On MRI, the tumor was hypo- to isointense on T1-weighted images and hyperintense on T2-weighted images compared with the normal gray matter, and was strongly enhanced with gadolinium. The tumor was first treated with fractionated conventional external-beam radiation (5120 cGy in 16 fractions over a 4-week period), resulting in a slight decrease in size of the tumor. For a definite diagnosis and mass reduction, surgery was performed using an interhemispheric translamina terminalis approach, resulting in a partial removal of the tumor due to profuse bleeding. Histological diagnosis was hemangioblastoma. Hemangioblastomas of the third ventricle are extremely rare and have not been specifically discussed. We describe the detailed clinicopathological features of the present case together with the possible explanation for the development of this tumor in this rare location.