OBJECTIVE: The usage of mechanical ventilator has been an issue in advanced stage of most neuromuscular diseases. The patients experience hypoventilation symptoms and usually die from pulmonary complications at last. Besides traditional invasive mechanical ventilation, non-invasive intermittent positive pressure ventilation (NIPPV) has provided an alternative treatment option. We evaluated the effects of NIPPV. METHOD: We applied NIPPV method to the patients with advanced neuromuscular disease who were hospitalized due to ventilatory failure, who visit our outpatient clinic due to hypercapnic symptoms, or who showed hypercapnia on a routine follow-up. To evaluate ventilatory status, blood gas tensions were analyzed by the arterial blood gas analysis and/or pulse-oxymeter and capnometer. Overnight pulse-oxymeter monitorings were done whenever necessary. RESULTS: Thirty patients were managed with NIPPV successfully. In five cases, invasive IPPV with tracheostomy at admission was switched to NIPPV. Three patients who had been intubated to receive IPPV were transferred to NIPPV without being tracheostomized. CONCLUSION: NIPPV can be used safely and effectively as an alternative method of ventilatory support for the patients with advanced neuromuscular disease who show ventilatory failure. It would relieve symptoms and signs of hypoventilation and prevent the acute respiratory muscle decompensation, if applied before overt ventilatory failure.