Objective　To investigate the early diagnosis and treatment of pleuropulmonary blastoma in children. Methods  The progress of pleuropulmonary blastoma from type 1 to type 3 was retrospectively analyzed. Results  The male infant had no obvious abnormality in chest X-ray at 18 days. At one year old, X-ray film of the chest showed cystic lesions near hilum of the left lung (about 5 cm × 4 cm × 4 cm). The congenital cystic adenomatoid malformation was considered and an operation resection was suggested. However, it had been rejected by his parents. At 3 years and 2 months old, chest X-ray and CT showed left lung had a solid cystic lesion, the volume was significantly enlarged, the back side was solid and the upper part had a large gas cavity. Two months later, the patient had asthmatic suffocation. The chest CT showed the tumor was completely solid. Pleuropulmonary blastoma type 3 was considered. The patient received the conventional chemotherapy after operation excision, and was followed up for 1 year. No recurrence was observed after the treatment. Conclusions  Type 1 pleuropulmonary blastoma and congenital cystic adenomatoid malformation are hard to be differentially diagnosed. Early diagnosis and treatment of type 1 pleuropulmonary blastoma can improve the prognosis.