Pleuropulmonary blastoma (PPB) is a kind of rare malignancy which often occurs in children and is related to dysplasia. It always involves in pleura and lung. PPB is misdiagnosed easily because of its special pathophysiological characters and complex biological behavior. Early detection and correct treatment are very important for thoracic surgeon to cure PPB. This paper reviews the advance of pathophysiological characters, molecular genetic characters, clinical manifestation, clinical diagnosis and differential diagnosis, and treatment and prognosis of PPB.