Cowden's Disease is an autosomal dominant genodermatosis associated with abnormalities of the breast, thyroid gland, gastrointestinal tract. We describe a 23-year-old girl who was diagnosed with this disease when she consulted for evaluation of a multinodular goiter. Further investigations revealed breast lesions, gastric polyposis and parotid cancer. Recognizing Cowden's disease is important for prompt screening for malignancies. Skin lesions are markers of precancerous development.