The causes of syncope are diverse and extensive; carotid body tumors are an extremely rare cause of syncope. These rare neoplasms represent less than 0.5% of all head and neck tumors. The authors present a case of a woman with syncope who was found to have a right-sided carotid body tumor. After surgical resection was performed, she did not have any additional syncopal or near-syncopal events. The authors provide a review of the literature on the natural history, presentation, and preferred management of carotid body tumors. With modern diagnostic tools and treatment options, most patients with this diagnosis can expect to recover fully.
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