Familial adenomatous polyposis is a rare genetic disease characterized by the development of more than a hundred adenomatous polyps in the colorectal area, as well as by extracolonic manifestations. Without treatment, this inherited disease, usually transmitted by autosomal dominant inheritance, predisposes to colorectal cancer. Treatment must be preceded by counseling about the nature of the syndrome and by recommendations for the optimal management and surveillance of the disease. Currently, prophylactic surgical therapy is imperative. However, the type of surgical technique used depends mainly on the severity of the polyposis phenotype, the age of the patient at diagnosis, and a series of special clinical circumstances. Lifetime follow-up of all patients is required. This article reviews the main studies published on familial adenomatous polyposis in order to provide an update on the most appropriate management of these patients.