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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that results in diffuse muscle atrophy, which invariably involves the respiratory muscles. Most patients die of respiratory causes, including hypercapnic respiratory failure and pneumonia. Whether educating patients about pulmonary involvement, diagnosing respiratory muscle weakness, treating respiratory problems, or counseling patients about end-of-life decisions, pulmonologists can play an integral role in the care of patients with ALS. Serial evaluation of pulmonary function with spirometry is a basic element of ALS care. Complicated presentations may require more extensive evaluations, including supine spirometry, maximal inspiratory pressure, and arterial blood-gas analysis. Proper respiratory support of ALS patients can improve survival and quality of life. Available treatments include noninvasive positive pressure ventilation and mechanical insufflation-exsufflation. Tracheostomy ventilation should be included as a potential treatment option for ALS. While very few ALS patients opt for tracheostomy and long-term mechanical ventilation, for select patients, this can provide years of support with a preserved quality of life. Pulmonologists' experience with mechanical ventilation and critical care medicine provide unique expertise for discussing end-of-life issues such as hospice care, tracheostomy, and withdrawal of care. This review provides a general overview of ALS care and describes the pulmonary evaluation and treatment of patients with ALS.