Peutz-Jeghers syndrome(PJS) is autosomal dominant genetic disease. Mucosa pigmentation and gastrointestinal polyps are the main clinical manifestations in PJS patients. The etiology is complex and unclear and may be closely related with the liver kinase B1, fragile histidine triad, and p53 genes. PJS gastrointestinal malignant polyps may form. In addition, PJS can also be associated with other tumors. Mucocutaneous melanin spots are unique characteristics of PJS in patients. Patients often receive treatment in stomatology departments, and treatment methods are surgery, freezing, dermabrasion, power dry, and laser treatments. Gastrointestinal polyps are removed by surgery. PJS patients should be treated as soon as to prevent cancer.