Patients with neuromuscular disorders are rare challenges in daily anaesthetic practice and intensive care. With their inherited impairment of the musculoskeletal system, cardiopulmonary involvement and complications, these disorders require careful preoperative evaluation and preparation, avoidance of certain anaesthetic agents, improved perioperative monitoring of vital functions, and careful postoperative care, including physiotherapy and pain control. Currently, new anaesthetic agents, techniques and surgical procedures are being introduced that may benefit many of these patients. The armamentarium of invasive and noninvasive diagnostic procedures for many neuromuscular disorders is also developing, especially in the genetic field. Anaesthetic and surgical risks and the possibility of increased or maintained quality of life should at all times be carefully weighed against the hazards of major surgery and prolonged intensive care.