Gastrointestinal polyposis with orocutaneous hamartomas (Cowden's disease).

A patient with Cowden's disease and multiple gastrointestinal polyps is presented. An association of gastrointestinal polyposis with this syndrome is made and the histopathological features are described. The cliniical features of Cowden's disease along with its familial tendency are discussed. Emphasis is placed on the importance of considering this diagnosis when evaluating patients with multiple gastrointestinal polyposis. Screening of these patients and their families for the associated malignancies should be performed.