Peutz-Jeghers syndrome(PJS)is a rare autosomal dominant genetic disease that often poses a threat to the life of patients due to acute and chronic complications or various cancers.In recent years,with the deepening of under-standing and clinical practice of PJS at home and abroad,significant progress has been made in the diagnosis,monitor-ing,and treatment of this disease.Traditional Chinese Medicine has also demonstrated obvious clinical effects through syndrome differentiation and treatment based on the etiology and pathogenesis of polyps,patient constitution,and dis-ease characteristics.However,there is currently no consensus on the diagnosis and treatment of PJS in China.Therefore,this article reviews the clinical manifestations,diagnosis and differential diagnosis,clinical monitoring and treatment of PJS,aiming to improve clinicians'understanding and diagnosis and treatment abilities of PJS,explore effective and safer targeted drugs combining traditional Chinese and Western medicine,and provide references for the development of con-sensus and medication suggestions for the diagnosis and treatment of PJS in China.