Oculopharyngeal muscular dystrophy is an autosomal dominant transmitted condition seen mainly in French Canada. The largest number of publications on these patients concerns a Quebec family whose descendants have spread throughout the United States. Families of different ethnic origins have also been reported from around the world, although there is no evidence that the neuromuscular disease reported is the same, despite the similarity of the syndrome. When severe oropharyngeal dysphagia results, these patients can significantly benefit from a cricopharyngeal myotomy.