A case of neuroectodermal tumor, a malignant schwannoma, of the cervical vagus nerve is presented. It had been previously misdiagnosed and was a recurrence. A review of the literature reveals this to be the sixth such case reported. The difficulties in establishing a diagnosis and the principals of adequate therapy are discussed; these include wide surgical resection and postoperative radiotherapy to the tumor bed. The correct recognition of their malignant potential is essential for long-term disease-free survival.