Colorectal cancer (CRC) is rare in young adults. It presents more frequently with stage 3 or 4 disease, underscoring its relevance in this population. Prognosis, matched for stage of disease at presentation, is likely similar to that in older adults, although survival is clearly lower for the youngest subgroups within this population. This article reviews the literature on the etiology, presentation, treatment, and outcome of CRC in young adults. New chemotherapeutic regimens have demonstrated survival benefits and the introduction of new biological agents has offered ways to control metastatic disease that may eventually show promise in the treatment of earlier-stage CRC. The benefit of these newer agents in young adults is assumed but currently unproven. Molecular genetic studies are increasing the understanding of the pathobiology of CRC and may ultimately allow at-risk patients to be identified at an earlier stage.