The incidence of carcinoma of the small intestine is very low in children, and it is also known that the prognosis of this tumor in children is very poor. A case of adenocarcinoma in a 13-year-old girl arising in the jejunum is herein reported. The girl underwent a curative resection and is free of recurrence or metastasis 12 months after operation. This particular case is reported here with a review of the literature.