Bilateral carotid body tumors are quite rare neoplasms. The coexistence of bilateral carotid body tumor, thyroid gland adenoma and thymoma has not yet been reported in the literature. We report on a 59-year old male who presented with a bilateral cervical mass. After the standard diagnostic procedures and surgical removal the diagnosis of a carotid body tumor was confirmed. On physical examination, the carotid body tumor is usually palpable as a firm, painless cervical mass. Even though carotid body tumors are slow-growing, and from a histological point of view benign, they sometimes attain massive size and therefore compress the surrounding tissue and create clinical pressure symptoms. A hereditary-familial tendency of these tumors has been noted. A common neuroectodermal origin is proposed as an explanation for the coexistence of the carotid body tumor and multiple endocrine tumors.