Cronkhite-Canada syndrome is characterized by generalized gastrointestinal polyposis, ectodermal changes, and the eventual development of diarrhea and weight loss. The pathogenesis of the disease is unknown, and there is no established therapy. The disease has a poor prognosis because of malnutrition resulting from altered absorption in the gastrointestinal tract. We experienced a case of a 56-year-old female with Cronkhite-Canada syndrome. After enteral nutrition and administration of prednisolone for 3 months, clinical improvement was noted with cessation of diarrhea, increased serum protein, disappearance of pigmentation, and regrowth of the scalp hair, finger- and toenails, Endoscopy showed resolution of the gastrointestinal polyposis. So we report here a case of diffuse gastrointestinal polyposis which has been in remission with steroid therapy, (Korean J Gastrointest Endosc 2001;23:113-117)