Carotid body tumor is the most common paraganglioma in the head and neck region. It is arising from carotid body which is one of the paraganglion in the intimate adherence and infiltration to the important carotid arteries. Till now there has been three cases reported in the Korean literature of our field. The two cases were treated with ligation of common carotid artery, so one of them was lost. Preserving carotid arterial flow is of extreme importance during surgery of this tumor. Recently we experienced a case of huge carotid body tomor classified into group III, treation with end in end anastomosis of carotid artery. So we report herein our case with a brief review of literatures.