The authors discuss the epidemiology, histology, symptomatology, diagnosis and therapy of carotid body tumors, basing their discussion on three personal observations and on a case of cervical dermatocyst which showed clinical and radiological data consistent with diagnosis of a glomus tumor. Chemodectomas of the neck are more often seen in the carotid body and are considered histologically benign, although they might induce local or distant metastases. Clinical presentation is often non-specific and may only consist in a slowly growing mass in the higher jugular-carotid region. Among the various imaging techniques (CT, MR), supra-aortic selective angiography, including digital subtraction techniques, is now considered the most reliable tool, while ultrasonography and gammagraphy with 99mTc-gluconate or 131I-MBG are recommended for screening and family testing purposes. Biopsy is to be avoided as it is very likely to cause massive bleeding and because of the high percentage of false negatives. Surgery is the choice treatment, and lateral cervicotomy is the recommended approach for exeresis of the mass, while radiotherapy should be planned in patients with precise contra-indications for surgery and when the tumor mass has already reached the skull base. Pre-operatory selective embolization is still controversial.