Primary adenocarcinoma of the jejunum which accounts for only approximately 3% of all gastrointestinal tract malignancies, is distinctly unusual. The rarity and the non-specific symptoms of this cancer, which are the major factors contributing to its poor prognosis, make the diagnosis difficult. As the prognosis of primary adenocarcinoma of the jejunum, once metastasized, is poor, a greater awareness of the possibility of a jejunal cancer must accompany aggressive diagnostic and surgical procedure. We report our experience of a child with primary adenocarcinoma of the jejunum with a brief review of the literature.