Carotid body tumors are among the most difficult technical problems with which the surgeon must deal. A personal review of 15 tumors occurring in 12 patients is reported with emphasis placed on the relationship of these tumors to multiple endocrine adenomatosis (MEA), their familial and bilateral occurrence, and their unusual locations in the neck. Idiosyncracies of clinical behavior in terms of diagnosis, sites of origin, arteriography, and natural history is examined in depth through a specific patient study. The surgical treatment of these tumors is described which includes a frank discussion of complications and pitfalls.