Sporadic spinal extramedullary hemangioblastomas of the conus medullaris are extremely rare. We present the case of a 40-year-old male with symptoms of severe back pain and monoradiculopathy. The magnetic resonance imaging (MRI) revealed an intradural extramedullary tumor attached to the conus medullaris. Total tumor removal was achieved via a typical posterior midline approach through laminectomy of L1 and L2 vertebrae, resulting in complete resolution of the preoperative symptoms. The histological examination was consistent with hemangioblastoma. To the best of our knowledge, this is the fifth case reported in the literature. We performed a brief literature review outlining the mainstay of diagnosis and therapeutic approach to these challenging lesions.
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