A case of Peutz-Jeghers polyposis associated with duodenal carcinoma but without cutaneous pigmentation was presented. Duodenal carcinoma is the most commonly associated neoplasm. The presence of vitiligo in this patient is coincidental. Of 14 reported cases of Peutz-Jeghers syndrome associated with intestinal carcinoma, in only two was the tumor shown to originate in the polyp. In our case, too, we believe the carcinoma developed independently.