We report the case of a 12-year-old boy, diagnosed of Klippel Trenaunay Weber syndrome, with hemangiomas and venous varicosities in the right leg, who complaint about incidental hernatochezia. In the colonoscopy some violet and plain angiodisplastic lesions like in the skin, were observed in rectum and distal sigmoid colon. We think this case is interesting, because of the unusual afectation of the gut in this disease and the importance of considering the endoscopic procedures in the evaluation of these patients.