Cowden disease is a rare genetic disorder characterized by the presence of multiple hamartomas in the skin, thyroid, breast, nervous system and gastrointestinal tract. Mucocutaneous lesions are the most constant and characteristic finding. Breast and thyroid neoplasms (benign and malignant) develop in up to two thirds of patients. Inverted follicular keratosis as the presenting feature of Cowden disease is rare as the disease is usually suspected by the appearance of multiple facial trichilemmomas, oral mucosal papillomatosis and acral keratoses.