Surgical treatment of the craniovertebral junction (CVJ) requires excellent management by the anaesthetist. Patients undergoing this type of surgery have a wide range of concomitant diseases. Therefore, before proceeding to CVJ surgery, it is recommended to analyse the clinical aspects of the patient that could complicate the outcome of the surgical procedure.In this paper we aim to establish what constitutes the best surgical and anaesthesia management of these patients. We consider airway management, trying to identify the gold standard for the patient. We also consider the most appropriate intraoperative approach to guarantee the best management of the patient.