Neuromuscular diseases frequently affect the respiratory system by impairing function of the mechanical respiratory pump that can lead to progressive hypoventilation, atelectasis, pneumonia, respiratory failure, and even death. Recognition of respiratory impairment can be difficult due to subtle signs and symptoms; therefore, the clinician must maintain a high index of suspicion in all patients with neuromuscular disease. Invasive and noninvasive mechanical ventilation, as well as a variety of respiratory therapy techniques, can be used to prevent these complications.