Noninvasive ventilation (NIV) has been widely used to decrease the complications associated with tracheal intubation in mechanically ventilated patients with neuromuscular diseases in acute respiratory failure. However, nasal ulcerations might occur when masks are used as an interface. Helmet ventilation is a possible option in this case. We describe two patients with acute respiratory failure due to Duchenne muscular dystrophy who developed nasal bridge skin necrosis during NIV. Helmet pressure support ventilation caused significant patient-ventilator asynchrony, leading to NIV intolerance. Thus, biphasic positive airway pressure delivered by helmet was applied, which improved gas exchange and patient-ventilator interaction, allowing successful NIV.