Skeletal and cardiac muscle involvement is a recognized feature of the muscular dystrophies. Visceral smooth muscle involvement of the gastrointestinal tract is not as well appreciated. Gastrointestinal symptoms may herald the onset of a muscular disorder and may be the predominant feature of the disorder. In some instances, smooth muscle dysfunction may be the only clinical manifestation of the disease. The gastrointestinal manifestations of the various muscular dystrophies are reviewed. Clinical, radiographic, and histologic features are discussed. Further study of the histopathology and pathophysiology of visceral smooth muscle involvement in the muscular dystrophies will have a substantial impact on treatment that, to a large extent, remains empiric.