Summary Summary: The great variety of rare neuromuscular diseases (NMD) is associated with specific anaesthesia- related risks that involve decreased cardiac and respiratory function, adverse reactions to different anaesthetics and, as a consequence, unexpected severe anaesthetic incidents like malignant hyperthermia (MH) and MH-like reactions including rhabdomyolysis and asystole. MH is a hereditary metabolic myopathy triggered by volatile anaesthetics and succinylcholine. In the MH crisis an excessive increase of free intracellular calcium ions induces a hypermetabolic syndrome with tachycardia, hypercapnia, acidosis, skeletal muscle rigidity, hypoxaemia and fever. The mortality rate of more than 60% in untreated cases can be reduced to nearly zero by an early and consequent therapy including the administration of dantrolene. Clinical diagnosis is more difficult in abortive MH than in the fulminant crisis. Whenever trigger substances are used in anaesthesia, it must be ensured that diagnostic measures such as blood gas analyses, determination of endtidal CO2 concentration, creatine kinase, electrolytes, core temperature and pulse oximetry can be performed at any time. Moreover, a sufficient amount of dantrolene must be available The trigger agents of MH can also induce severe MH-like incidents in other neuromuscular diseases, often even prior to clinical signs and diagnosis. Specific risks associated with the different patient groups include myotonic reactions caused by succinylcholine in patients with myotonia and paralyses in patients suffering from dyskalaemic paralysis. The use of "non-triggering" agents for induction of anaesthesia is appropriate for all neuromuscular diseases. Myocardial and respiratory insufficiency in progressive NMD require careful preoperative diagnostic investigation, invasive intraoperative monitoring, and postoperative intensive care; they also need to be given critical consideration in any determination of the clinical indication for an anaesthetic and operative procedure.