Chemodectomas (CBTs) are the most frequently encountered tumours at the carotid bifurcation. Even if rare and commonly benign, their development close to the head and neck structures is often source of morbidity by compression and infiltration. Therefore, in order to avoid permanent neurologic or vascular complications these infrequent lesions need to be early removed. The total excision may be technically challenging and requires extensive expertise in surgical neck anatomy and a cooperative multidisciplinary approach. Almost a quarter of CBTs infiltrate carotid vessels so vascular expertise is pivotal in their resection. The present study aims to give an overview of vascular specificity and procedures required during surgical excision of such tumors to assist and guide treating physicians who encounter CBTs. This overview will particularly emphasize current therapeutic options: we discuss the referral criteria that should guide the decision about what type of preoperative care and surgical procedure should be offered to the affected patients. We also specify the epidemiologic data, screening recommendations and outcomes achieved with the different therapeutic approaches. The clinical, operative and follow-up data about a case of a Shamblin III carotid body tumour surgically removed by a multidisciplinary team at our Institution are also reported.