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Carotid body tumors may occur sporadically (90%) or in a familial pattern (10%). One third of the patients with familial disease have bilateral tumors. We report the case of a patient with bilateral carotid body tumors and a strong family history of such tumors. Details of his evaluation and treatment are reviewed. Relatives of patients with carotid body tumors should be examined carefully, and any suspicious neck mass should be appropriately investigated.
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