Neuromuscular disorders is a general term that encompasses a large number of diseases with different presentations. Progressive muscle weakness is the predominant condition of these disorders. Respiratory failure can occur in a significant number of diseases. The use of devices to assist ventilation is quite frequent in these types of patients. Noninvasive ventilation can be applied by various means, including nasal, oronasal, or facial masks. Masks, type bilevel positive airway pressure, continuous positive airway pressure, and similar are generally supported on the maxilla. Oral health in pediatric neuromuscular diseases has some peculiar aspects that we must consider in these patients’ follow-up. Based on a clinical case, this chapter provides a better understanding of these patients. It will focus on the oral and maxillofacial morphological alterations and preventive measures and strategies for oral pathologies management in this population. Despite always aiming at esthetics, treating these patients should always prioritize the possibilities of improving the oral and general functions of the body.