The diagnosis and management of carotid body tumors are discussed, including the importance of good arteriography which allows visualization of all major feeding vessels. These include the carotid bulb, internal and external carotid arteries and the often neglected but important vertebral and ascending cervical arteries. Emphasis is placed on the importance of establishing tourniquet control of the common, internal and external carotid arteries. Surgeons removing carotid body tumors should be experienced in arterial wall repair by means of vascular shunts, end-to-end arterial anastomosis, and vein graft replacement of a segment of arterial wall.