STUDY DESIGN A report of a case of metastatic spinal neurofibrosarcoma. OBJECTIVE To document metastatic neurofibrosarcoma as a cause of spinal cord compression and to review the literature. SUMMARY OF BACKGROUND DATA Three previously reported cases of metastatic neurofibrosarcoma of the spine were reviewed. METHODS The patient's clinical record and radiologic investigations as well as the result of a search of the English literature are reported. Magnetic resonance images, computed tomographic scans, and histology photomicrographs are displayed. RESULTS Paraparesis developed in this patient, due to a posterior extradural thoracic spinal cord compression by a neurofibrosarcoma believed to be metastatic from a neurofibrosarcoma of the femoral nerve. CONCLUSIONS Malignant spinal metastasis remains a rare complication of neurofibromatosis, with a very poor prognosis.