Carotid body tumor is a rare benign tumor arising from the paraganglionic tissue of neural crest. Surgical management remains the prefered treatment. Large carotid body tumors frequently encircle the internal carotid and external carotid arteries, and extensive bleeding often complicates the resection, increasing the risk of carotid artery rupture and damage to major cranial nerves. Recent improvements in surgical techniques and selective embolization have lessened the risks of surgical excision, decreased blood loss, and diminished the time required for resection. The review of literatures revealed a few cases of the carotid body tumor with papillary carcinoma of the thyroid. We report a case of the huge carotid body tumor with papillary carcinoma of the thyroid, which was removed by 4 times of preoperative embolization and transcervical approach.