Abstract: Small bowel adenocarcinoma (SBA) is a relatively uncommon neoplasm with poor prognosis. However, the incidence rate of this condition increases. SBA is usually diagnosed at the late stages, and the majority of patients present with the advanced stage. Data are limited when making decisions for treatment because of the lack of randomized trials for SBA. Radical surgery is considered necessary when possible. Adjuvant chemotherapy is predicted to be beneficial, but this procedure has not yet been investigated through randomized trials. Platinum-based chemotherapy is apparently the most effective treatment regimen used in retrospective tri -als for advanced SBA. Targeted therapies, such as those against the angiogenetic pathway or the epidermal growth factor receptor pathway, have not yet been established for this type of cancer. This article reviews the progress in the diagnosis and treatment of SBA.