Introduction:
Carotid body tumors (CBTs) are certainly unusual. They are vascular lesions originating from paraganglionic cells, located at the common carotid artery (CCA) bifurcation. They represent less than 0.5% of head and neck tumors, approximately 1-3 cases per million. Malignant CBTs are extremely rare; in the literature, published rates on average are < 10%. The diagnostic criteria for malignancy should be based on the finding of distant metastasis. Due to its unpredictable nature and its malignant potential, diagnosis before metastasis and complete surgical resection are the keys to a favorable prognosis.
 Case Report:
Given little experience in CBTs, its biology and treatment remain uncertain. We present the case of a 48-years-old patient, with a mass on the left side of the neck that was found to be a vast CBT with suspicious histopathology. Its size, rare location, pathologic findings, and management strategy applied for its treatment, illustrate an unusual case that highlights the importance of its publication.
Conclusions:
CBT is rare, but subject to cure lesion if resected without metastatic or residual disease. This is why surgery should be performed whenever possible and why it is so necessary to study this pathology thoroughly and to take it into account in the differential diagnosis.