Abstract
Cowden Syndrome, a rare autosomal dominant condition with PTEN gene mutations, generates abnormal proliferation of several tissues and higher risk to develop malignancies. The diagnosis is based in clinical manifestations according to the International Cowden Consortium. A 27-year-old male patient presented multiple papules on the upper and lower alveolar ridge, face and hands. In 2010, verrucous lesion was removed from the inferior lip being diagnosed as verrucous carcinoma. The papules on the gingiva were diagnosed as fibro-epithelial hyperplasia and the association with clinical findings suggested a syndromic condition. The patient was referred to a gastroenterologist and his endoscopy revealed numerous polyps in the esophagus and stomach and positive urease test. Thyroid ultrasound showed benign nodules in both lobes. Laboratory tests presented no alterations. The findings met the criteria being diagnosed Cowden Syndrome. The dentist has an important role in the diagnosis of systemic diseases that manifest in the oral cavity.
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