A 30-year-old man presented with a subacute course of myelopathic signs and symptoms. Magnetic resonance imaging demonstrated an epidural mass lesion of the spinal canal at the cervicothoracic junction causing compression of the spinal cord. Laminectomy with resection of this lesion revealed a large varix with acute and chronic thrombus. Postoperatively, an improvement in neurological function occurred. Spinal epidural varicosities have been reported as an etiological factor in lumbar and sacral radiculopathies. This is the first reported case of spinal cord compression in association with spinal epidural varices. The diagnosis, pathophysiology, and management of this disorder are presented.