AbstractBackgroundCarotid body tumor (CBT) is a rare neoplasm that has been increasingly studied during the last decades; nevertheless, it continues to be a topic of controversy. This review aims to provide an update on the general features of CBT and particularly review different treatment strategies and primary outcomes.MethodsData for this literature review were identified by PubMed, Scopus, and Medline. 93 articles from the initial search were included, as well as 28 relevant studies utilizing the snowballing method; totaling 121 articles about CBT.ResultsMain features such as anatomy, embryology, genetics, clinical presentation, and diagnosis of CBT are presented, followed by evidence of different treatment strategies such as radiotherapy, preoperative embolization, vascular resection, and vascular reconstruction. Main complications are also discussed.ConclusionThis review summarizes the most critical aspects regarding CBT. Future studies should compare different treatments to attain the best surgical results with lower morbidity rates.