Introduction: Carotid body tumor is a rare benign tumor originating from cells of neural crest tissue. It could present with Horner's syndrome. This tumor is rare and comprises 0.5% of all neoplasms. A familial incidence with autosomal dominant transmission has been reported. I have a series of five patients from a known family with one death during surgery because of carotid artery rupturing, others were diagnosed by color Doppler US and dynamic CT scan. Generally, it is sporadic and more frequently seen in high-altitude living people.Sex incidence is approximately equal, "little bit more frequent in the female".A characteristic saddle deformity is seen in invasive angiography.Symptoms are nonspecific; such as, headache, dizziness, tinnitus, loss of hearing acuity, hoarseness, vocal cord or hypoglossal nerve paralysis and syncope. Horner's syndrome is due to sympathetic chain involvement.Three stages or types of disease have been described by Shambling and colleagues First, minimally involved internal carotid artery "sub-adventitial"Second, partial incorporation of the sheath of internal carotid arteryThe third type is encircling of internal carotid artery with dense adherenceImaging: High frequency, high-resolution ultrasonography and color Doppler study are necessary to see blood flow in the artery, bifurcation widening and blood flow in the tumor (low resistance).Spiral dynamic X-ray CT: Spiral CT with MPR'S and 3D-angiography is the best imaging to show tumor vascularity and the internal carotid lumen.DSA or invasive angiography is used for preoperative embolization (larger than 2 cm) just before surgery (gold standard).MRI with dynamic and without GD and MRA could replace X-ray CT scan.Treatment is surgery, embolization and radiation therapy.Ninety-seven patients have been reported from Iran by Mohammad Taghi Salehian as an original article.Rare statements: A malignant unilateral chemodectoma may appear and metastasize to the bone in 6-12.5% of the cases. The abdominal wall is another rare location of metastasis.A sex-linked genetic trait is possible. All bilateral tumor cases are benign. 90% of tumors are unilateral and 10% are bilateral. Sporadic forms are more common than the inherited variety.65% of head and neck Paragangliomas are carotid body tumors.Classification is sporadic, familial and hyperplastic.The common age involvement is the fifth decade, but in MENS (Multiple endocrine neoplasia) syndrome, it develops in younger patients.Carotid body tumors have been reported in siblings of a Turkish family together with the involvement of adrenal pheochromocytomas.Differential Diagnosis: The main differential diagnoses are lymphadenopathy, bronchial cleft cyst, metastatic carcinoma, lymphoma, neurogenic tumors, salivary gland tumors, aneurysm, giant cell arteries, hematoma, carotid calcification or stone.