Hemangioblastoma of the conus medullaris with MRI has not been reported before. A 75-year-old man had a history of falling due to weakness of his left lower limb. MRI revealed a well-defined oval mass in the conus medullaris. The tumor had an isointense signal relative to spinal cord on T1-weighted images, hyperintense signal areas intermixed with punctate spots of hypointensity on T2-weighted images, and heterogeneous obvious enhancement on gadolinium-enhanced T1-weighted images. Associated abnormally tortuous vessels were noted in the dura proximal to the tumor. Histological findings were compatible with the diagnosis of hemangioblastoma. Hemangioblastoma should be included in the differential diagnosis in patients with an enhancing tumor and adjacent engorged vessels of the spinal cord.