Respiratory failure is an inevitable problem in the progression of some neuromuscular diseases. Chronic muscle diseases that commonly involve the respiratory system include metabolic myopathies, congenital myopathies, congenital myasthenia gravis syndrome, and some myotonic dystrophies. Pompe’s disease is autosomal recessive and is caused by mutations in the gene encoding acid α-1,4 glucosidase (GAA). The diagnosis can be confirmed by muscle pathology, blood enzymatic tests and genetic screening, and up to 70% of respiratory muscles are involved [1-2]. The only effective enzyme replacement therapy for Pompe disease has not been introduced in mainland China and is very expensive. Therefore, for now, once the diagnosis of Pompe disease is established, treatment options are very limited. The main goal of treatment is to prolong the patient’s life and improve the quality of life, and the monitoring and maintenance of the respiratory system to avoid premature and rapid progression of respiratory failure becomes crucial. In 2013 Ambrosino N et al [3] proposed a more reasonable model for respiratory monitoring in patients with Pompe disease. And they suggested several issues that often stump specialist physicians. 1. To what extent is noninvasive respiratory support recommended for patients with respiratory involvement? After all reversible factors not related to Pompe disease (infection, heart failure, severe electrolyte disturbances, etc.) have been corrected, hypoventilation symptoms (fatigue, dyspnea, morning headache) are present and any of the following are met: (1) significant CO2 retention during the day (PaCO2 > 50 mmHg); (2) decreased oxygen saturation at night (SaO2 < 88% for > 5 minutes); (3) FVC < 50% of predicted value or MIP < 1500pxh2o. 2. When should the patient be advised to use invasive respiratory support? When non-invasive respiratory support is not adequate, such as when respiratory support is required for more than 20 hours per day; when sputum cannot be effectively expelled even with an assisted device. Pompe disease is one of the rare genetic disorders that are often first diagnosed in internal medicine or respiratory medicine because of significant respiratory involvement. Early recognition of Pompe's disease by more internists and active interdisciplinary collaboration with neurologists after the initial diagnosis will allow for better diagnosis and treatment. In this era of MDT (multidisciplinary treatment), it is believed that more patients with Pompe disease will receive more benefits.