Three members of a family with Peutz-Jeghers syndrome had intussusception due to intestinal hamartomas. In addition, the grandfather had carcinoma of the jejunum, and the granddaughter had benign ovarian sex cord/stromal tumor causing sexual precocity. Benign and malignant tumors, not often associated with Peutz-Jeghers syndrome, pose a further threat in addition to the gastrointestinal bleeding and obstruction that often complicate this inherited syndrome.