In this seminar we describe the critical care management of patients with chronic neuromuscular diseases (cNMD). Determination of the acuity of the critical illness and trajectory of illness in the setting of cNMD is necessary to guide decision making. Systemic complications of critical illness, cardiac support needs, and peri-intubation considerations may be affected by underlying diagnosis. Mechanical ventilatory support, whether noninvasive or invasive, requires redefinition of the goals of ventilation on a patient-by-patient basis. Mode and approach to invasive ventilation and liberation to noninvasive ventilation versus tracheostomy have limited evidence, but potential clinical approaches are reviewed.