Carotid chemodectoma (CC) is a very rare neoplasia. It originates from Type 1 main cells of the carotid body and affects both sexes almost in the same proportion (mostly in the fourth and fifth decades). It can be on a familiar basis (5-10%) and in these cases it is more frequently multicentric, being sporadically found in association with other paragangliomas. CC is a slow-growing neoplasia, locally aggressive and it can give metastases to regional lymph nodes and surrounding anatomical structures. Once surgically removed, it can reoccur in a small percentage of patients. Early diagnosis is of the utmost importance and surgeon's skill is fundamental as well as awareness of the real nature of the disease. Surgical treatment of CC is difficult and demanding, for the close relation of CC to the neighbouring neurovascular structures. The authors report a case of CC radically operated on. Clinical parameters are analysed as well, and Literature is reviewed.