We report a case of a 41-year-old man with a primary thoracic spine paraganglioma presenting with spinal cord compression and distant metastasis. Paragangliomas arising within the spinal canal are rare and when they occur are usually found in the intradural, extramedullary compartment and in the lumbosacral region. Thoracic paragangliomas are very rare, with only five prior cases reported in the literature and with distant metastasis seen in only one of these. In this case, the tumor was entirely extradural and produced extensive sclerosis of the surrounding vertebrae. The clinical, radiographic, and pathologic features of this case are detailed and the relevant literature is reviewed.