Peutz-Jeghers syndrome is a dominantly inherited polyposis syndrome characterized by hamartomatous polyps of the gastrointestinal tract and pigmented lesions of the skin. Since its description, much debate has centered on the true malignancy risk of Peutz-Jeghers syndrome including malignancy within and outside the gastrointestinal tract. Rare tumors such as adenoma malignum of the cervix occur in Peutz-Jeghers syndrome. Likewise, more common cancers occur at younger ages in Peutz-Jeghers syndrome. Children may manifest symptoms of this disease including gastrointestinal complications and malignancy. The gene associated with this condition remains unidentified.