Amyotrophic lateral sclerosis (ALS) is a devastating motor neuron disease causing progressive paralysis and eventual death, usually from respiratory failure. Treatment for ALS is focused primarily on optimal symptom management because there is no known cure. Respiratory symptoms that occur are related to the disease process and can be very distressing for patients and their loved ones. Recommendations on the management of respiratory insufficiency are provided to help guide clinicians caring for patients with ALS.