The Peutz-Jeghers (P-J) syndrome is a rare condition and only few cases have been reported in Taiwan. We analyze the clinical data of 10 cases of P-J syndrome collected from 1985 to 1994 in Tri Service General Hospital (TSGH). Mucocutaneous pigmentation and gastrointestinal (GI) hamartomatous polyps, the characteric findings of P-J syndrome were presented in all these ten cases. The polyps in these patients with P-J syndrome were usually multiple and were found throughout the GI tract, mainly in the small intestine. Five cases received surgical intervention because of complications including GI intussusception, bleeding and obstruction. Two of total 37 resected hamartomatous polyps displayed mucosal dysplastic change. Another case developed bilateral infiltrating duct carcinoma of breast. One case reveived three times exploratory laparotomies for P-J syndrome related GI complications in four years.