Ancient Schwannoma is a type of peripheral neurogenic tumor formed by the Schwann cells presenting mainly as a benign and asymptomatic lesion. he neurilemmoma tumor appears in diferent sites and, in cases of cervical location, can mimic a carotid body tumor. Herein we describe a clinical case of a 51-year-old woman with latero-cervical swelling. A contrast-enhanced computed tomography scan revealed a classical wineglass image mimicking a carotid body tumor. During surgery the tumor appeared connected to the cervical sympathetic trunk without carotid involvement. he histological exam conirmed the nature of the mass which consisted of an ancient schwannoma. A subsequent systematic review of the literature on ancient schwannoma incidence and treatment conirms it being a benign and rare lesion primarily treatable with open surgery.