Hereditary colorectal cancer conditions are associated with a very-high life-time risk of colorectal cancer and require an intensive endoscopic surveillance programme. All colorectal lesions identified during follow-up should be endoscopically removed. Colectomy should be considered when these lesions are not amenable to endoscopic resection because of their morphological characteristics and/or their multiplicity. In fact, colectomy or coloproctectomy is indicated in virtually all patients with classical form of familial adenomatous polyposis as they develop hundreds of adenomatous polyps during their second decade of live. In any case, it is recommended that surgical decisions are discussed in multidisciplinary teams and that surgery is performed in highly specialized centres.