OBJECTIVE AND IMPORTANCE: Approximately 20 to 27% of neck masses in the parapharyngeal space may be benign nerve sheath tumors. Cervical sympathetic chain schwannomas or vagal nerve schwannomas are most common. We report a patient with a cervical sympathetic chain schwannoma. CLINICAL PRESENTATION: The patient was a 47-year-old Caucasian man with a 7-month history of a painless, enlarging right neck mass. No hoarseness or Horner's syndrome was found preoperatively. Formal head and neck examination in the otolaryngology department revealed no vocal cord dysfunction. INTERVENTION: By use of a standard right carotid incision, a tumor was identified growing from the cervical sympathetic chain posterior to the carotid bifurcation. A gross total resection was performed and a section of the cervical sympathetic chain was sacrificed. CONCLUSION: Cervical sympathetic chain schwannomas are unusual tumors that require microneurosurgical resection for cure. We discuss the differential diagnosis, evaluation, surgical management, and pathological characteristics of such tumors and review the literature.