Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder characterized by hamartomatous gastrointestinal polyposis and mucocutaneous pigmentation. We report here the case of a 28-year-old young woman presenting with PJS revealed by an acute small bowel obstruction at the MED VI University Hospital of Marrakech. Surgical exploration revealed hamartomatous polyps necessitating segmental resection. Anatomopathological examination confirmed the diagnosis. Postoperative recovery was uneventful. Surveillance revealed no abnormalities.
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