Respiratory complications are a major cause of morbidity and mortality in pediatric neuromuscular disease. In adults with acute or chronic respiratory failure, noninvasive positive pressure ventilation (NIPPV) is superior to standard therapy in terms of preventing intubation and reducing mortality. Moreover, domiciliary ventilation with NIPPV in children can prolong life and optimize the quality of life by allowing patients to eat, speak and sleep well. But the role of NIPPV in pediatric patients with respiratory failure is not well established. We report the case of a pediatric spinal muscular atrophy patient who was managed by using NIPPV for acute hypercapnic respiratory failure at a pediatric intensive care unit and by nocturnal hypoventilation later at home. In this case, NIPPV restored normal blood oxygenation, removed excess blood carbon dioxide, and normalized the work and pattern of breathing.