Carotid body tumors are rare, slow-growing, hypervascular neuroendocrine tumors. Although these tumors are benign neoplasm, they also have a tendency to malignant transformation. Complete surgical excision is the gold standard therapeutic modality for the treatment of carotid body tumors. Early surgical removal is recommended to prevent the development of larger and more advanced tumors, which are associated with higher morbidity and mortality. In this report, we presented three cases of carotid body tumor which were successfully treated with complete surgical excision, and reviewed the current literature. Furthermore, it was emphasized the necessity of early surgical management regardless of patient age and tumor size.