A 6-year-old girl was admitted to the emergency department with abdominal pain and bilious vomiting of 3 days in duration. Abdominal ultrasound examination showed an 8-cm-long intussuscepted intestinal segment with a target sign. There was a 26 x 28 x 23 mm nonperistaltic anechoic cystic mass suggestive of a duplication cyst. At laparotomy, the ileocecal region was normal with many enlarged lymph nodes from which biopsies were taken. There was a 20-cm-long intussuscepted segment at the proximal ileum close to the jejunum. After manual reduction, a 2-cm-long edematous segment resembling a duplication cyst served as the lead point. The segment was excised, and a primary bowel anastomosis was performed. She was discharged on the fifth postoperative day. The histopathologic examination revealed that the excised segment contained a gastrointestinal stromal tumor measuring 2.5 cm, with a mitotic rate of 2 to 3 mitoses per 50 high-power fields (low-risk group) showing an infiltrative growth pattern. On immunohistochemistry assay, some of the tumor cells were CD117 and CD34 positive, whereas all of them were smooth muscle actin and S-100 positive but CD10 negative. Staining index with Ki-67 was 5%. Surgical margins were free of tumor. The lymph nodes showed reactive hyperplasia. She was referred to the pediatric oncology department for further evaluation. Gastrointestinal stromal tumors are common in adults and may lead to intussusception. To the best of our knowledge, this is the first childhood case of gastrointestinal stromal tumor causing jejunoileal intussusception in the literature. (c) 2012 Elsevier Inc. All rights reserved.