A 62 year old gentleman, recently diagnosed with amyotrophic lateral sclerosis, was admitted due to acute-on-chronic hypercapnic failure. He was successfully managed with noninvasive bilevel ventilation and was discharged home under the recommendation to continue the use of noninvasive ventilation during sleep. Three months later however, his condition had deteriorated and he was using mechanical ventilation for >18 hours/day. His vital capacity had dropped from 1.3 to 0.7 L and he had a peak cough flow (PCF) of 50 L/min. To defer tracheostomy the patient was started on mouthpiece ventilation in the assist volume control mode, with a tidal volume of 0.9 L, a zero PEEP and a back-up rate of 14/breaths/ min. The patient was taught how to utilize mouthpiece ventilation in order to perform the “air-stacking” maneuver, which increased his PCF to 200 L/min. The management of this case suggests that the use of noninvasive respiratory support strategies can obviate tracheostomy and improve cough efficacy in selected patients with neuromuscular diseases.