Pulmonary involvement is common in patients with muscular dystrophy, and routine screening for evidence of respiratory muscle dysfunction should be performed. Testing for respiratory muscle strength, as well as an assessment for sleep-disordered breathing, should be obtained in consultation with a specialist. Adequate airway clearance and correction of impaired ventilation are the key components of pulmonary management in this group of patients. Also, special emphasis on airway clearance is recommended during upper respiratory infections, as well as in the postoperative period. Early initiation of ventilatory support has been shown to result in longer life expectancy.