Children with neuromuscular diseases present a wide range of clinical manifestations and clinical implications for the anaesthesiologist. Neuromuscular diseases in children affect muscle strength by either directly weakening the muscle fibrils or indirectly by a degenerative nerve supply and weak neuromuscular junction. Of the more than 200 neuromuscular disorders known, the vast majority are genetic in origin. This review focuses on four of the more common neuromuscular disorders with emphasis on their pathophysiology and clinical implications for anaesthesiologists: malignant hyperthermia, the muscular dystrophies (Duchenne's, Becker's, and Emery-Dreifuss), mitochondrial disorders, and cerebral palsy.