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 Ki-67 (75%) 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.