Familial adenomatous polyposis (FAP) is a hereditary disease characterized by presence of numerous colorectal adenomas. It often exposes its carrier to absolute risk of colorectal cancer, but also to other extracolonic tumours (especially to duodenal cancer and desmoid). Screening and surveillance of FAP patients leads to reduction of colorectal cancer incidence and mortality. Colonoscopy/lower endoscopy and esophagogastroduodenoscopy (including use of side-viewing endoscope) are the principal examinations. Colectomy is the standard therapeutic procedure, but endoscopic therapy plays relevant role both in upper and lower gastrointestinal tract. Recent international guidelines and some new tools for severity classification enable effectively reduce the mortality related to this disease by individualized patient management. Key words: colorectal cancer - familial adenomatous polyposis.