Acute respiratory failure (ARF) is a life-threatening condition that often results from the acute onset of neuromuscular disease (NMD) and often coexists with other cardiorespiratory conditions. For more than two decades, existing studies have shown that non-invasive ventilation (NIV) is the main ventilatory support and provides a good clinical outcome in ARF with various conditions, but its use in NMD patients with ARF is still limited. In patients with ARF, NIV can be initiated in fully awake patients with hemodynamically stable, without upper airway obstruction, and airway secretions can be overcome. Furthermore, expiratory positive airway pressure (EPAP) and inspiratory positive airway pressure (IPAP) settings with backup rates are recommended. Additionally, some studies have reported that the application of NIV in NMD with ARF can also be beneficial as a weaning strategy. Hence, further studies need to be conducted to generate evidence regarding the role of NIV in NMD patients with ARF.