Small bowel neoplastic disease is a rare but dreaded occurrence in Crohn's disease (CD) and the diagnosis is often disguised by nonspecific and varied presenting symptoms mimicking active or obstructive CD. As such, the diagnosis is all too often delayed, typically detected at a late stage, and with a poor prognosis. CD has become a well-recognized risk factor for the development of small bowel adenocarcinoma. The data, however, are limited and based on case reports, retrospective studies, and review of the literature.