Unlike the colon and rectum, the small intestine is associated with a very low rate of tumor occurrence. Adenocarcinomas represent the most frequent of these rare digestive tumors and are often fatal as a result of tardy diagnosis. Regardless of the stage, surgery usually remains the cornerstone of small bowel adenocarcinoma therapy. Because of the rarity of the disease, very few significant clinical trials have identified any efficient nonsurgical treatment; however, recent data indicate these tumors might be sensitive to chemotherapy alone or in association with radiation therapy. Conversely, a great deal of progress has been achieved in diagnosis of the tumor, whether by adaptation of existing techniques or development of new ones. We reviewed the clinical aspects of this rare but aggressive disease, focusing on new diagnostic procedures as well as on recent advances in their therapeutic management.