This is a report of a rare case of Peutz-Jeghers syndrome in a 13 years old boy with chief complaints of melena and intermittent abdominal pain since 3 years of age. He has multiple small melanin pigmentations in the face, lower lip, and left buccal mucosa and numerous small and large polyps in the stomach, jejunum, ileum, colon, and rectum by roentgenologic studies. Hereditary features, mucocutaneous pigmentations, clinical manifestations, pathologic features, roentgen features, and differential diagnosis, and treatment of Peutz-Jeghers Syndrome are discussed with a review of the literature.