The normal function of the palate and the pharynx during speech involves the closure of the velopharyngeal orifice during the majority of speech sounds. In order to properly direct articulated speech out of the mouth, it is necessary for there to be a complete seal between the mouth and nose; otherwise, hypernasality will result from a failure to close the velopharyngeal orifice (called velopharyngeal insufficiency, or VPI). VPI is typically associated with cleft palate, but it can occur secondary to trauma, neurologic disorders such as cerebral palsy, and is a fairly common problem in children with primary myopathies. This report describes the sample of patients with myopathies referred to a single craniofacial center specifically because of VPI. Within a total patient population of more than 6,000 cases, approximately 2,000 have been specifically referred with the complaint of VPI and/or hypernasal speech. The largest percentage of these cases had overt or submucous palatal clefts. Nearly all of these cases have been treated surgically with pharyngeal flaps. An additional 1,000 cases have been referred with dentofacial abnormalities which often require reconstructive orthognathic surgery. Patients with primary myopathies often have both dentofacial abnormalities and VPI secondary to their disease. In a retrospective review of diagnoses from the craniofacial patient population, 28 were identified with primary myopathies. Of these 28 cases, all of whom had dentofacial disproportions, 13 had VPI. The male to female ratio was 13:15 with a mean age of 10.5 years. Diagnoses were confirmed by biopsy in 23 cases and consisted of 17 with myotonic dystrophy, 4 with nemaline myopathy, 1 with Duchenne's muscular dystrophy, and 1 with facioscapulohumeral dystrophy. Pharyngeal flap surgery was recommended and implemented in 4 cases, all having successful outcomes without complications, including 2 with myotonic dystrophy, 1 with nemaline myopathy and 1 with facioscapulohumeral dystrophy. Though surgery and anesthesia are often regarded as risky options for patients with myopathies, we have found that successful outcomes can be achieved in select cases without complications. Therefore, physicians managing patients with myopathies should be aware of VPI as a common complication and that treatment options are available for improving speech.