Peutz-Jeghers syndrome (PJS) is a rare disease of autosomal dominant inheritance, which is characterized by multiple hamartomatous polyps and mucocutaneous pigmentations. Multiple hamartomatous polyps in the gastrointestinal tract are the hallmarks of PJS, and occur most commonly in the small intestine. Complications induced by polyps include colicky abdominal pain, bleeding, bowel obstruction; rectal prolapse of polyps, and intussusceptions. A male patient visited our emergency room with colicky abdominal pain. The patient was diagnosed small intestine intussusceptions with multiple polyps. We report this case with a review of the literature.