Although 3% to 30% of lesions in von Recklinghausen disease undergo malignant transformation, malignant transformation of benign solitary schwannoma is extremely rare. We reported a case of recurrence and malignant transformation in a benign intraosseous schwannoma arising in the cervical spine of a 44-year-old man. The patient presented giant tumor in the C3 vertebral body with aggressive, expansile, and osteolytic destruction and relapsed 2 years after surgical resection and spinal reconstruction. Clinical data, radiologic characteristics, surgical management, histopathologic and immunohistochemical features were noted in the duration of follow-up. The local recurrence, nuclear pleomorphism, epithelioid differentiation, a small number of positive S-100 protein-staining cells, and especially the high percentage of positive cells with p53 (80%) and proteins support the aggressive nature of the lesion in malignant transformation of benign intraosseous schwannoma in the cervical spine. Immunohistochemistry would be useful as an ancillary technique in diagnosis. It is our practice to suggest that such case has to be carefully resected and the patient followed up. An intraosseous schwannoma is the infrequent occurrence and uncommon lesion. The mandible is the most common site. 6 The incidence of the intraosseous schwannomas is less than 0.2% in primary bone tumors.7 Cases of the osseous changes in the spinal schwannomas are observed in almost one third of cases (6 of 17) where, radiographically, 1 or 2 vertebral bodies can be seen eroded with welldefined marginal sclerosis. 4 The most common imaging findings of extradural spinal schwannomas involving bone include pedicle erosion, vertebral body scalloping, and widening of the neural foramen.8 Based on the radiologic findings, Sridhar et al 9 proposed a classification system of spinal schwannomas as types I to V, in which type V of spinal schwannomas was defined as a giant invasive tumor in spinal intraosseous schwannomas. In a few patients the tumor presents as an expansile vertebral body lesion in the cervical spine, as reported in the literature. 6,[9][10][11][12] Although primary malignant peripheral nerve sheath tumors (MPNSTs) of spine with or without the intraosseous involvement in association with von Recklinghausen's disease (neurofibromatosis type I) have been reported in the thoracic and lumbar spine, 12-15 malignant transformation of benign solitary schwannoma is extremely rare. 16,17 Woodruff et al 17 described 2 patients with such changes involving a finger and pelvis and reviewed 7 patients with malignant transformation. Malignant transformation of benign schwannoma has also been occurred in the vestibular nerve, 18 retropharyngeal space, 19 neck, 20 and intrathoracic extrapleural space 21 without an association of von Recklinghausen disease. The possibility of occult malignancy could also be associated with a previous radiation.18,22 To our knowledge, malignant transformation of benign intraosseous schwannomas in the spine has not been reported yet.W...