Introduction: Carotid body tumor, also known as a chemodectoma or carotid body paraganglioma that arises from the paraganglion cells of the carotid body. It is located at the carotid bifurcation with characteristic splaying of the ICA and ECA. Case: A 34-years-old woman, presented with complaints of gradually progressive swelling right lateral upper neck of 2 years duration. Examination of the neck revealed a hard, tender swelling measuring 5*3 cm. Contrast neck CT revealed Bilateral carotid space hypervascular tumor. A diagnosis of CBT that removal of carotid body tumor right side was planned then Removal of CBT was performed. The internal carotid artery was damaged during surgery, so reconstructive surgery was performed using the internal jugular vein. There are no ischemic changes in the brain after surgery. Biopsy: Epitheloid cell arranged nests, consistent with paraganglioma. Immunohistochemical confirmed paraganglioma. Consclusion: CBT is a rare tumor, but the most common type is paraganglioma of the head and neck. Surgery is performed depending on various factors, including the patient's age, comorbidities, location and size of the tumor, and previous cranial nerve defects.