This article provides a critical review of the clinical presentation, pathology, genetics and management of the polyposis in childhood starting from the isolated juvenile polyp to the hereditary polyposis syndromes. These include the juvenile polyposis syndrome, Peutz-Jeghers syndrome, PTEN hamartoma tumor syndromes and familial adenomatous polyposis (FAP). These conditions typically outbreak in the second decade of life but early
detection and proper screening and surveillance can minimize the risk of intestinal and extracolonic cancers. While juvenile polyp could be easily removed, the amartomatosis syndromes will require a specific endoscopic surveillance and a periodic polypectomy. In the case of FAP, the paediatrician has a decisive role in defining the colonscopic follow-up and the timing of the total colectomy.
