Background: Carotid body tumors (CBTs) are rare neoplasms originating from the carotid body. A comprehensive synthesis of the available evidence is essential to enhance the understanding of this condition among clinicians and researchers. Purpose: This review aims to provide a comprehensive overview of CBTs, encompassing their epidemiology, pathophysiology, clinical manifestations, current treatment modalities, and associated complications. Research Design: A narrative literature review was conducted, synthesizing information from relevant studies identified through a systematic search of electronic databases. Study Sample: The review included studies focusing on human subjects with carotid body tumors, encompassing case reports, case series, cohort studies, and relevant review articles. Data Collection and Analysis: The literature search was primarily conducted in the PubMed database. The search strategy utilized a combination of keywords and Medical Subject Headings (MeSH) terms related to “carotid body tumors,” “chemodectoma,” and “paraganglioma.” Retrieved articles were screened based on titles and abstracts, and full texts of relevant studies were reviewed for data extraction. Data on epidemiology, pathogenesis, clinical features, treatments, and complications were then thematically analyzed. Results: Epidemiological data indicate a slight increase in the global incidence of CBTs, although they remain relatively uncommon. The pathogenesis involves factors such as genetic predisposition, chronic hypoxia, and geographical influences. Clinical presentation is variable, commonly featuring neck masses, carotid sinus syndrome, and voice changes, depending on tumor size and location. Primary treatment options include surgical excision, radiotherapy, and embolization, with the choice of modality influenced by patient age, tumor size, and location. While surgery is often the preferred approach, it carries risks of complications such as cranial nerve injury, stroke, and hemorrhage. The review also identifies and discusses rare complications, including baroreceptor reflex failure. Conclusions: A thorough understanding of the multifaceted aspects of CBTs is crucial for refining diagnostic and therapeutic strategies. This integrated knowledge is expected to contribute to improved patient survival and quality of life.