Respiratory complications are a major cause of morbidity and mortality in the pediatric neuromuscular diseases. Weakness of the muscles of respiration results in shallow breathing and ineffective cough, making patients vulnerable to atelectasis, pneumonia, and tracheal obstruction by retained respiratory tract secretions. Assessment of the risk of respiratory complications includes evaluating the patient's history and respiratory physical examination and measuring pulmonary function and gas exchange. Treatment options include methods of assisted cough and mechanical ventilation. This review emphasizes the use of noninvasive respiratory aids to optimize duration and quality of life in these potentially fatal diseases.