Velopharyngeal incompetence (VPI) has been associated with neuromuscular disorders. Only 4 patients with myotonic dystrophy (MD) who underwent pharyngeal flap elevation for VPI have been reported in the literature. In 3 patients, surgery preceded the diagnosis of MD. Cardiorespiratory complications characterized the postoperative period of 3 patients. The authors present 3 patients with VPI and an established diagnosis of MD (by molecular genetics) who underwent pharyngeal flap elevation. The operation resulted in a major improvement in speech in all patients, although some relapse was noted later in 1 patient. Contrary to previous reports, none had peri- or postoperative cardiorespiratory complications. MD, although an uncommon etiology, should be considered in cases of late-onset VPI. Owing to differences between the authors' findings and previous reports, additional studies are needed before final conclusions can be reached regarding the benefit and safety of pharyngeal flap surgery in MD patients. At present, MD should not be considered a contraindication for this procedure, although close perioperative monitoring is indicated.