The Peutz-Jeghers syndrome is an autosomal dominant disorder characterized by hamartomatous polyposis of the gastrointestinal tract, melanin pigmentation of the skin and mucous membranes, and an increased risk for cancer. The incidence of surgical complications in these patients is relatively rare, and correlates with the size and location of the polyps. Herein we report the case of a 27-year-old woman presented with episodes of abdominal pain, abdominal distention and intermittent vomiting. Moreover, multiple pigmentation of the mouth was also noted. A preoperative diagnosis of a double jejunal intussusception and jejunal occlusion was based on the findings of small bowel enema and computed tomography. The diagnosis was confirmed at laparotomy.