Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease. Most patients die from respiratory complications. Fortunately, there are a growing number of treatment options that can improve both survival and quality of life for patients with ALS. This review discusses the respiratory evaluation and treatment of patients with ALS, about which a great deal is known. It also includes material on related problems, such as speech and swallowing difficulties and end-of-life care.