In 1921, Peutz reported the families with pigmented spots on the lips, oral cavity, and distal portion of the extremities, and digestive tract polyposis over three generations, later in 1949, Jeghers et al. introduced the definitive descriptive reports and discussion from the references about 10 cases including his self-tested case. As a result, since then this is a disease which comes to be called Peutz-Jeghers syndrome. The diagnosis of this disease is based on,(1)an autosomal dominant trait,(2)brown and black pigmented spots on the lips, oral cavity, and distal portion of the extremities,(3)digestive tract polyposis. It is considered that the cases recognized all of(1),(2),(3)are classified into the complete type, and the cases recognized two symptoms out of three are classified into the incomplete type.