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The Peutz-Jeghers syndrome first described by Peutz in1921 (1) in a Dutch family and later by Jeghers in 1949(2), is an inherited syndrome of hamartomatous gastroin-testinal polyps and mucocutaneous pigmentation.Recently the molecular basis of the syndrome was descri-bed as a germline mutation in the STK11/LKB1 geneon chromosome 19 p13.3 (3). Several studies have shownan important neoplastic potential for gastrointestinal andextraintestinal tumors. Possible surgical complications aregastrointestinal obstruction, bleeding and perforation.The obstructive ileus can be caused directly by a volu-minous polyp or indirectly by means of intussusception(4). We report the case of a 27-year-old woman withan intussusception of the small bowel due to Peutz-Jeghers syndrome.