The main factors of modern perioperative care of the craniovertebral junction surgery include a comprehensive approach to the patients, including a thorough cardiorespiratory, neurophysiological, and metabolic assessment, intraoperative monitoring of spinal cord function, safe airway management, and judicious use of fluids and blood transfusions. Admission in PICU shortly after the CVJ surgery is mandatory to ensure haemodynamic and respiratory stability and to recognize postoperative complications. Anticipating complications in order to achieve an early treatment and adverse event prophylaxis can contribute to reduced morbidity and mortality and increased patients' safety. Multidisciplinary management of perioperative patient care and careful pain control is mandatory in order to improve the outcomes.