Familial adenomatous polyposis (FAP) is a rare, hereditary disease characterized by the presence of 100 or more adenomas distributed throughout the colon and rectum. If untreated, colorectal cancer develops in almost 100% of FAP patients. As prophylactic treatment, proctocolectomy with ileal pouch-anal anastomosis remains the surgical treatment of choice. High rates of postoperative complications, however, have been reported with this procedure, including bowel dysfunction, incontinence, and reduced female fecundity. Some novel strategies for preventing hereditary colon cancers have been reported. This review summarizes alternative treatments, including the laparoscopic approach, chemoprevention, endoscopic management, and subtotal colectomy combined with endoscopic treatment, for prophylaxis of colorectal cancer in FAP patients.