A case of Peutz-Jeghers syndrome (PJS) associated with intestinal intussusception accompanied by ultrasonographic abnormalities of the thyroid gland and testicles in a 12 year-old boy is described. Patients with PJS are known to be at increased risk of developing tumors at both intestinal and extra-intestinal sites. Recently, one of the PJS genes has been mapped to chromosome 19pl3.3. This is the first report of a case of PJS associated with thyroid abnormalities similar to the ones seen in patients with Carney complex. This report adds an example of predisposition of PJS to extra-intestinal tumors and indicates the importance of life-long observation for malignancy.