Cowden's disease (CD) is a rare syndrome (little more than 100 cases r
eported in the literature) [1], inherited as an autosomal dominant tra
it with an incomplete penetrance and variable expressivity [2] and cha
racterized by multiple hamartomas and neoplasms of ectodermal, mesoder
mal and endodermal origin, predominantly localized to skin, breast, th
yroid gland and gastrointestinal tract. On this subject we describe a
case of a patient with oral papillomatosis, acral keratoses, intestina
l polyps and goiter.