Abstract: Objective :To make sure that Peutz-Jeghers' Syndrome is a familial disease. Methods :Clinical data from 1 case of Peutz-Jeghers' Syndrome were analyzed retrospectively. Results :This family includes 6 patients.One was suspect;2 patients underwent Intussusception reduction and partial bowel resection;1 underwent partial small bowel resection;1 underwent partial small bowel resection and partial large bowel resection;1 underwent pancreatoduodenectomy because of carcinogenesis of Duodenal polyps;2 underwent Endoscopic eleectrocoagulation removal.Fanally,2 patents died of lung cancer,one died of liver metastasis. Conclusion :Peutz-Jeghers' Syndrome is a familial disease,which might be changed into carcinoma and accompanied with the malignant tumors of other organs.Patients should be monitored for all their life.The best choice is the resection of polyp through endoscope.Concise operation will provide good conditions for post-operative fellow-up.