Carotid body tumor is rare and benign neoplasm that is originated from the extra-adrenal paraganglion system. It is almost always nonfunctional tumor. Diagnosis is generally confirmed by an angiography that shows a vascular tumor enlarging the space between the internal and external carotid artery. Surgical removal is the mainstay of management, and identification and preservation of neural and vascular structure is required. Preoperative embolization for the reduction of the vascularity may be useful. The authors have experienced 2 cases of carotid body tumor, which were managed surgically. One of the two had bilateral tumor, which was removed by stage operation. We report two case of carotid body tumor with a review of literature.