Objective To analyse the clinical features of Peutz-Jeghers syndrome and its canceration, and to summarize the management and the follow-up strategy for patients with Peutz-Jeghers syndrome. Methods Clinical data of 30 patients with Peutz-Jeghers syndrome dating from October 1985 to September 2005 in West China Hospital of Sichuan University were analyzed retrospectively. Results Fourteen (46.67%) definite family histories of Peutz-Jeghers syndrome were found. Pigmentation of skin and mucosa, abdominalgia and hematochezia were major clinical manifestations of the syndrome. There were 18 patients (60.00%) complicated with intussusception and acute intestinal obstructions, 16 patients (53.33%) with gastrointestinal bleeding, and 6 patients (20.00%) developed malignancy. The frequent types of malignancy were carcinoma of small intestine (3 cases), colon carcinoma (2 cases) and gastric carcinoma (1 case) in order. The mean age of the canceration-diagnosed patients was 32 years old. The type of pathohistology of all the malignancy was poorly differentiated mucus adenocarcinoma. High-frequency endoscopic electroresection, orthdox polypectomy and enterectomy were the major means of treatment. Conclusion Patients with Peutz-Jeghers syndrome are at high risk of canceration at relatively early ages and usually the differentiation of the tumor is poor. Endoscopy should be performed regularly and the high-frequency electroresection is an effective therapy in disposing intestinal polyp. Screening can also improve the efficacy of Peutz-Jeghers syndrome.