Puetz-Jeghers syndrome involving appendix

Puetz-Jegher's syndrome is an autosomal dominant hereditary disease, which is characterized by hamartomatous polyposis and mucocutaneous pigmentation mainly over the circum-oral region. Patients with Peutz-Jeghers syndrome seek medical attention whenever there are complications such as intussusception, bleeding from the polyps, etc. Occasionally, gastrointestinal tract malignancies have been reported in Peutz-Jeghers syndrome. In this paper, we report a patient with Peutz-Jeghers syndrome who had multiple complications and polyposis involving the appendix, because involvement of the appendix is extremely rare in Peutz-Jeghers syndrome.