Children with severe neuromuscular disorders are at risk of restrictive lung disease. The frequency and severity of this complication in children with inherited neuropathies is unknown. We undertook a clinical audit aiming to understand the extent and spectrum of, and risk factors for, respiratory impairment in inherited neuropathies in children. Children with a genetically confirmed and/or clinical diagnosis of Charcot-Marie-Tooth disease were studied. Demographics, clinical severity of peripheral and axial muscle weakness, and symptoms and signs of respiratory muscle involvement were recorded in all cases. Respiratory dysfunction was assessed using the clinical history (number and severity of chest infections, symptoms of sleep-disordered breathing), pulmonary function testing and polysomnography. Respiratory involvement in inherited neuropathies in children may be more common and significant than has previously been recognized.