Schwannoma is a benign tumor of Schwann cell origin and may occur on any nerve covered by Schwann cells. Although approximately 25% to 48% of all Schwannoma occur in the head and neck region, the lesions originating from the cervical vagus nerve are extremely rare. We have recently experienced a case of huge Schwannoma arising from the cervical vagus nerve which was initially misdiagnosed as a huge goiter. We report herein the case with review of the literatures.