Background: Sclerosing epithelioid fibrosarcoma (SEF) is a rare sarcomatous tumor that presents infrequently as an osseous lesion of the spine. To our knowledge, few case reports exists regarding primary lesions of the lumbar spine with only one report indicating thoracic spine as a primary site. Case Description: We present a case of a 32-year-old female presenting with primary osseous SEF with complaints of axial and right sided thoracic back pain for 4 months. Imaging of the thoracic spine revealed a mass at T5 with vertebra plana and central spinal cord compression at T5-T6 without significant expansion of mass to soft tissues or adjacent vertebral levels. We performed a T5 corpectomy with posterior spinal instrumented fusion from T3-T7. Pathologic and immunohistochemical studies confirmed diagnosis of SEF. 1 year follow-up revealed no recurrence of disease and significant pain relief without neurologic dysfunction. Conclusions: In light of the extent of the vertebral body involvement, the severity of cord compression, and the patients associated neurologic symptoms, a T5 corpectomy with T3-T7 posterior fusion was performed. Given the nature of the spine, complete resection of these tumors is usually impossible and adjuvant radiation or chemotherapy may be necessary for resolution of disease.