Primary intraosseous malignant peripheral nerve sheath tumors (MPNST) are exceptionally rare; the case reported here represents the first documented example occurring in the spinal column. The tumor arose in the second cervical vertebra of a forty year old woman of Asian origin. She had no stigmata of von Recklinghausen's disease (VRD). Plain radiographs and magnetic resonance imaging showed an osteolytic lesion within the expanded body of the vertebra. Histology revealed spindle-shaped tumor cells with nuclei showing pleomorphism, pallisading and mitotic activity. The cells stained positively for S-100 protein. The patient underwent stabilisation of the cervical spine and tumor excision as a two-stage procedure. She died of pulmonary metastases one year later. We have reviewed the literature on the pathology of these tumors.