Familial adenomatous polyposis is a rare genetic disease characterized by the development of colorectal adenomatous polyps. Extracolonic digestive and extra-digestive manifestations can also appear. Inevitably, colorectal cancer occurs if a colectomy is not performed. Sulindac is an indolic non-steroidal anti-inflammatory indole drug which decreases colonic tumoral proliferation. The different trials published since 1983 have shown that sulindac caused regression of colorectal adenomatous polyps, but it does not affect the other manifestations of familial adenomatous polyposis. However, colorectal polyps recurred after cessation of this therapy; the effect of long-term sulindac therapy is unknown; and sulindac may cause, as a non-steroidal anti-inflammatory drug, digestive side-effects. Moreover, treatment with sulindac does not completely eliminate the risk of cancer. For patients with familial adenomatous polyposis, total colectomy and ileal pouch-anal anastomosis is the recommended procedure for most patients. However, sulindac is useful for patients who have had subtotal colectomy and ileorectal anastomosis, if these patients have only a few rectal stump polyps and accept regular and strict follow-up of the rectal stump. Sulindac is also indicated for patients who have not undergone colectomy because surgery is contraindicated or has been refused.