The authors extensively review the international literature on juvenile polyposis, with particular reference to the risk of malignancy in the various forms of the disease (colorectal vs. generalized, familial vs. sporadic). Sixty-eight out of a total of 412 patients presented adenomatous changes in the polyps. In addition, 68 cancers were found. The rate of degeneration was analysed for all variants of juvenile polyposis, but no statistically significant differences were detected. The authors conclude that juvenile polyposis should be considered as being as challenging to the surgeon as familial polyposis syndromes and outline protocols for the screening, endoscopic and surgical treatment and follow-up of these patients.