Small bowel cancers are rare, accounting for only about 6000 cases/year in the United States, approximately 25% of which are small bowel adenocarcinomas. Small bowel adenocarcinomas have traditionally been considered to be highly fatal due to their nonspecific presentation at the time of diagnosis, and to the lack of responsiveness to older chemotherapy regimens. However, that paradigm may be changing. Newer diagnostic techniques such as video capsule and double balloon enteroscopy may facilitate earlier diagnosis. In addition, modern chemotherapy regimens have produced improved response rates and survival rates, when compared to historical controls. Still, there remains great need for multi-institutional, cooperative group studies to define the optimal treatment of small bowel adenocarcinoma, both in the adjuvant and advanced/metastatic setting.