Schwannomas are rare and slow-growing neurogenic tumors for which surgery is the standard of care. However, the anesthetic management of these tumors can be challenging. This case report describes the anesthetic management of a patient who underwent carotid endarterectomy and excision of a presumed carotid body tumor. Histopathologic examination showed that the excised tissue was a schwannoma, which in this location can mimic a carotid body tumor. The relevant literature is also reviewed.