Peutz-Jeghers syndrome is a familial disease characterized by spots on the lips and intestinal polyposis. A review of the history and the anatomic features of the disease are presented. One of us (AJM) has the largest personal series in the literature with twenty patients in five generations. Pedigree charts and aspects relating to the development and interrelationships of spots and polyps are explained. A previously unreported instance of colon intussusception is presented along with a discussion of the clinical presentation and the malignant potential.

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