The carotid body is a small structure weighing 12 mg located in the adventitia of carotid artery bifurcation acting as a chemoreceptor. Carotid body tumour (CBT);formerly known as chemodectoma is a rare, highly vascular, mostly benign tumour arising from the paraganglia of carotid body;hence, the name (carotid paraganglioma). The high vascularity and proximity to cranial nerves and major vessels make this tumour a surgical challenge. Abundant literature has been written about CBT in the last century with a continuous debate regarding its etiology, natural history, biological behavior, proper technique of excision, and the morbidity and mortality associated with its resection. The purpose of this review article is to simplify understanding the basic and clinical aspects of this challenging neoplasm.