Gastrointestinal polyps are common during childhood and most often present with painless rectal bleeding. Most polyps occur as isolated colonic lesions and are not harbingers of an underlying genetic disorder, nor do they bestow a risk of gastrointestinal cancer. The astute pediatrician must be aware, however, that occasionally polyps may occur in the context of a genetic polyposis disorder characterized by an increase in the life-time risk of cancer in the gastrointestinal tract and other organ systems. This review outlines the major polyposis syndromes affecting children and highlights associated findings that might clue the alert physician to an underlying diagnosis. Recent developments in the understanding of the genetics of each disorder are emphasized.