Two cases of carotid body tumor (chemodectomas) have been reported and at this regard the international literature reviewed. Concerning the diagnosis selective angiograms represent the investigation of choice in clinical assessment and planning operative approach. Doppler-ultrasound results particularly useful in the follow up of patients after surgery. With advances in vascular technique, complete excision of the tumor has become possible and is now associated with minimal morbidity and mortality. Survival rate of resected patients is equivalent to that for sex age matched control subjects.