We present a case of synchronous bilateral carotid body tumor and vagal paraganglioma in a 49-year-old man who presented with 3 months history of tenderness and palpable neck masses bilaterally. An encapsulated mass which was thought to be a carotid body tumor and an incidental 3 cm x 2 cm, 5 cm x 1 cm, 5 cm mass which seemed to be originated from vagus nerve were dissected from the left side. Pathology revealed carotid body tumor and vagal paraganglioma. Six months after the first operation, the carotid body tumor on the right side was totally excised. A discussion of this case is followed by a review of the literature surrounding this rare clinic and pathological entity.