The authors describe a 14-year-old girl with Peutz-Jeghers syndrome who presented with progressive abdominal distention. Radiologic investigation revealed large polycystic tumors on both ovaries. Bilateral ovariectomy was performed and microscopic examination showed mucinous ovarian tumors. The authors discuss the increased risk for cancer in patients with the Peutz-Jeghers syndrome and review the different ovarian tumors associated with this syndrome. They also stress that these patients must be followed not only for gastrointestinal disease but also by regular gynecologic examination.