Background: Carotid body tumor is a hypervascular tumor with multiple feeding arteries and unique orientation at the carotid bifurcation. Although resection is a radical therapy for this tumor, complete resection is challenging. Materials and Methods: Articles reporting carotid body tumor treatment and surgical resection were reviewed including case-control series and review articles. Results: Selected reports were reviewed and discussed focusing on choice of treatment, surgical difficulties and preoperative embolization of feeding arteries. Conclusion: Multiple feeding arteries and adhesion of the tumor to the carotid arterial wall are causes of difficulties in carotid body tumor resection. The effectiveness of preoperative embolization remains controversial due to the varied situations in performing surgical resection among the institutions. However, perfect embolization and resection immediately after embolization reduce blood loss and operative time of surgery for carotid body tumor.Carotid body tumor (CBT) is a rare disease that originates from the paraganglion cells (paraganglioma) of the carotid body at the carotid bifurcation. The World Health Organization classification designated this tumor as malignant because it has a malignant potency and there is no requirement to distinguish benign from malignant features in the pathological findings of specimens (1).It is well-known that malignant tumors cannot always be identified by their morphological features in histopathological examinations; clinical findings such as metastatic activity can distinguish malignant tumors from their benign counterparts. Only clinical findings, such as lung, liver, or bone metastasis, indicate that a tumor is malignant. The slow-growing feature of CBT represents an almost benign character and embryonic origin plus germline mutation of this tumor. Its potential for metastatic activity highlights the need for surgical resection. Surgical resection of CBT by head and neck surgeons must be considered once a patient is referred to a hospital. However, in contrast to the slow-growing feature of this tumor, characteristic features, such as a rich vascular network of its capsule supplied by many feeding arteries, complicate resection (2).In recent years, it has been revealed by molecular biological studies that various types of gene alterations exist in the succinate dehydrogenase (SDH) gene family such as point mutations (3-5). Most patients with CBT were shown to have variants with germline mutations, such as SDHB and SDHD. Conversely, the idea of "hereditary paragangliomapheochromocytoma syndrome" has been frequently used to explain patients with familial paraganglioma, and it extends to patients with CBT who have a family history of the disease and gene alterations (6-8). Therefore, analyses of gene alterations are needed for patients with CBT. In addition, systemic diagnosis is needed to identify other types of paraganglioma, such as pheochromocytoma, since patients with SDH variants tend to have multiple paragangliomas (9).The Sha...