Twelve cases of Peutz-Jeghers syndrome in one family have been studied, and these are presented together with a review of the history and features of the syndrome. This syndrome was detected in seven of the ten siblings and in the father. One family member underwent repeated surgery after small bowel obstruction was diagnosed. In addition, multiple polyps were removed from the rectum. Rectal bleeding dominated the clinical picture in two patients. Three of the siblings had small bowel polyps, five of the siblings had polyps in the colon. A colonic polyp was also found in one patient who had a small bowel polyp. Two of the seven affected siblings and the father of the family had mucocutaneous pigmentation.

Keywords: intestinal polyposis, Peutz-Jeghers syndrome