Peutz-Jeghers syndrome (PJS) is characterized by the combination of gastrointestinal hamartomatous polyps and mucocutaneous pigmentation. Polyposis is most prevalent in the small intestine, but also presents in the stomach and large bowel in the majority of affected persons. The classic mucocutaneous stigmata include dark pigmented macules around the mouth, eyes, and nostrils, on the buccal mucosa, and in the perianal area, and hyperpigmented areas on the fingers. Affected females have increased risk to develop sex cord tumors with annular tubules (SCTAT), a benign ovarian neoplasm, as well as adenoma malignum of the cervix, a rare aggressive carcinoma. More infrequently, affected males can develop calcifying Sertoli cell tumors of the testes which may cause gynecomastia.