Velopharyngeal incompetence has previously been described in the late stages of myotonic dystrophy. However, we have seen three cases in which velopharyngeal incompetence was the presenting symptom. Despite different ethnic backgrounds, the patients shared a similar appearance, all three had the characteristic "myopathic facies," mandibulomaxillary disproportion, muscular weakness, and positive family history. Identifying a progressive neuromuscular disorder as the cause of velopharyngeal incompetence has important treatment and prognostic implications. Based on the experience described in this report, we feel that the velopharyngeal incompetence of the myotonic patient might best be managed nonsurgically.