An unusual case of genodermatosis with familial gastrointestinal polyposis, angiomatous malformation and ascites.

Gardner's syndrome is a familial adenomatous polyposis syndrome with extraintestinal manifestations, characterized by the coexistence of intestinal polyposis with an early risk of malignant degeneration and extraintestinal manifestations mainly involving the skin, eye, bone and thyroid. We describe an unusual case of intestinal adenomatous polyposis, retinal hypertrophy, fibromas of the skin, bone and thyroid tumors accompanied by congenital arteriovenous malformations with lethal complications.