Abstract: Familial adenomatous polyposis (FAP) is an autosomal dominant disease. Colonoscopy and genetic testing provide patients with an opportunity of early diagnosis and treatment. Genetic testing is the development direction of FAP diagnosis and the key to further study on its pathogenesis. Total proctocolectomy with ileal pouch-anal anastomosis (IPAA) and total colectomy with ileorectal anastomosis (IRA) is the main surgical approach in FAP treatment, especially the application of laparoscopy, and it provides new opportunities and challenges for surgeons. The proposal of new surgical procedures and their safety and effectiveness need to be verified in clinical studies and further improvement and development. The extracolorectal manifestations of FAP should not be neglected, especially duodenal and ampullary cancers and desmoid tumors, which have become important causes of death in patients. This review focuses on the diagnosis and surgical treatment of FAP.