First described in 1963 by Lloyd and Dennis [4], Cowden syndrome, or multiple hamartoma syndrome [1], is a rare autosomal-dominant inheritance genodermatosis [6], characterized by multiple hamartomas of ectodermal, mesodermal, and endodermal origins [4-6]. The phosphatase and tensin homolog (PTEN) gene negatively regulates cell proliferation and cell cycle progression [1]. This evidence concerns the gene PTEN and Cowden disease.