Abstract: Carotid body tumors (CBT) are classified as chemoreceptor tumors. There is a familial genetic predisposition, and the occurrence of CBT is related to mutation of the SDH gene. Most of the cases are benign; the rate of malignancy is less than 10%. The criterion for a diagnosis of malignancy is the presence of disease in a regional lymph node or distant metastasis. The diagnosis may be based on the results of careful clinical examination and special imaging techniques, including ultrasound- B examination (color Doppler imaging), CT and MRI/MRA, as well as DSA. The DSA examination is considered the gold standard for diagnosis of CBT. Active surgical treatment is to be carried out once the diagnosis is made. At present, radiotherapy remains the treatment of choice for patients with carotid body tumor. The purpose of this article is to summarize and review the recent developments in the diagnosis and management of CBT.