Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with no known cure. Involvement of muscles of the respiratory system, the inspiratory, expiratory, and upper airway muscles, is the major cause of morbidity and mortality. Dyspnea, swallowing difficulties, sialorrhea, and impaired cough are all symptoms that can be palliated with pharmacological and nonpharmacological methods. Noninvasive positive pressure ventilation (NPPV) in particular is a technique to assist ventilation that not only relieves dyspnea and ameliorates respiratory failure but may also extend the lives of patients with this disease. It should be offered to all ALS patients with a forced vital capacity of less than 50%.