Noninvasive positive pressure ventilation (NPPV) is used for respiratory support in a number of diseases causing acute or chronic respiratory failure. We describe a novel use of NPPV to provide respiratory support during sedation for percutaneous placement of a gastrostomy tube in a patient with Duchenne muscular dystrophy (DMD). The patient had severe respiratory insufficiency, progressive dysphagia, and undernutrition. In addition to the case in this report, we have used NPPV to provide respiratory support to DMD patients during five other gastrointestinal endoscopies without complication. The technique is highly labor intensive and requires physicians and respiratory therapists familiar with NPPV. The primary risk associated with this technique is lack of definitive airway protection during the procedure, which must be balanced against the risks of intubation in an anesthetized patient with neuromuscular disease. The potential benefit to selected patients is substantial, such as initiation of gastrostomy tube feeding in our patient, with subsequent improvement in his quality of life and nutritional status.