In the final stage of amyotrophic lateral sclerosis (ALS) the majority of patients develop chronic respiratory failure due to respiratory muscle weakness. The interaction between the patient with ALS and the physician should be characterized by continuous communication, especially with respect to the prospect of ventilatory failure and for support. The patient and his family must be informed thoroughly about the natural history and the prognosis of ALS, depending on the individual disease process. Already in the early stage of the disease coping strategies should be discussed so that imminent respiratory emergencies can be handled. If ALS patients are not informed about the acute respiratory insufficiency they run the risk of having to be intubated and mechanically ventilated over a long term. If dyspnea and hypersecretion dominate the final stage of ALS, the therapeutic strategy consists of the administration of morphine, insufflation of oxygen and bronchoscopic suction. Mechanical ventilation should only be initiated in the exceptional case. However, if dyspnea occurs in the early stage of the disease, when there is no bulbar paralysis and peripheral muscle function is intact, then noninvasive mechanical ventilation via mask may improve the quality of life substantially. Nevertheless, invasive mechanical ventilation via a tracheostomy should be avoided.