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Three cases of Peutz-Jeghers polyposis with carcinoma of the digestive organs are studied. Although mucocutaneous pigmentation was not present in two of the three patients, the features of intestinal polyposis are consistent with those of Peutz-Jeghers syndrome. One patient had a carcinoma of the pancreas and the other two had carcinomas with colonic Peutz-Jeghers polyps. Previous reports on carcinomas associated with Peutz-Jeghers syndrome are reviewed. An unusual location in the gastrointestinal tract, together with occurrence at an early age, characterize the carcinoma in Peutz-Jeghers syndrome. In Japanese patients, the large bowel is the site of the greatest number of carcinomas. On the other hand, Western patients showed a relatively even distribution. A possible surveillance protocol for early detection of gastrointestinal carcinoma in patients with Peutz-Jeghers syndrome is discussed.