A 17-year-old female was admitted to our hospital with abdominal pain, vomiting and abdominal swelling. On the same day, we performed computed tomography (CT) and diagnosed it as small intestine ileus, so an ileus tube was inserted. As both small bowel X-Ray examination through the ileus tube and single balloon enteroscopy showed a stenosis of the upper jejunum, we took a biopsy specimen. Histological findings showed that the stenosis was an adenocarcinoma. We diagnosed it as primary small intestinal cancer and performed a surgical operation one month later. We will report on this rare case of juvenile small intestinal cancer, including some review of literature.