The Peutz-Jeghers syndrome, a condition characterized by harmatomatous polyps of the gastro-intestinal tract and mucocutaneous pigmentations, appears to be transmitted in an autosomal dominant fashion, but de nove cases have been reported without any suggestive family history. The mucocutancous pigmentations are usually found on and around the lips and buccal mucosa, fingers and toes. The harmatomatous polyps which are predominantly located in the small bowel, particu larly the jejunum but also can occur in the stornach, colon and rectum are not generally regarded to he prernalignant. But in patinents with this syndrome, increased rates of both intestinal and ext:raintestinal malignancies has been reported. The authors here present a case of clinical expcrience of a 33-year old male patients with Peutz-Jeghers syndrome who had an advanced adenocarcincma at the rectum near the rectosigmoid junction and metastatic hepatic cancer with review of literatures.