Colorectal cancer remains the second leading cause of cancer deaths in the United States, with an estimated 143,000 new diagnoses anticipated in 2012. The lifetime risk of any individual developing colon cancer is 5.1%. Often, the disease has no presenting symptoms, but can manifest through changes in bowel habits or appetite, vague abdominal pain, overt bleeding, occult blood loss, iron deficiency anemia, weight loss, or obstructive bowel symptoms. A significant number of afflicted individuals demonstrate a family history of colon cancer. Moreover, a small subset of these individuals will present with features of an established familial cancer syndrome. Careful integration of the family and personal history, physical examination findings, and endoscopic findings play a critical role in recognition and management of these high-risk individuals. Small bowel cancers remain rare, and account for less than 0.5% of all new cancers diagnosed. Malignancies of the small bowel include adenocarcinoma, lymphomas, carcinoids, and mesenchymal tumors. On occasion, cancers of the small bowel may also represent as a manifestation of a familial cancer syndrome.