Carotid body tumors are the most common type of paraganglioma of the head and neck. They are usually diagnosed in the 4th to 5th decade and have a female predilection. They typically arise at the bifurcation of the common carotid artery causing splaying of the internal and external carotid arteries. Malignant transformation has been reported in up to 36% of cases. Surgical excision is the treatment of choice.