Cowden syndrome (CS) is an autosomal dominant genodermatosis that frequently affects several tissues with hamartomatous growth. The oral cavity is quite commonly involved with papillomatous lesions, which can be crucial to early diagnosis of this disease. In this series, 10 patients with a great diversity of manifestations associated with CS are presented, in whom oral papillomatosis was a constant and relevant finding to establish the diagnosis of CS. The role of the dentist in recognizing the oral lesions, the other diagnostic criteria, the risk for the development of malignancies, and the importance of lifetime follow-up are discussed.1174E301E310