A case of Peutz-Jeghers syndrome associated with ovarian mucinous cystadenoma and ovarian sex cord tumor with annular tubules is presented. The sec cord tumor with annular tubules was described in 1970 by Scully, who recognized its striking association with the Peutz-Jeghers syndrome. This tumor is an almost constant finding in patients' ovaries with this disorder. Three cases of ovarian mucinous cystadenoma and sex cord tumor with annular tubules associated with Peutz-Jeghers syndrome were found in the literature. Our observation confirms that gynecologic abnormalities are an important manifestation of the syndrome and require careful surveillance.