Objective: To explore the clinicopathological features, diagnosis and treatment of type Ⅰ and type Ⅱ pleuropulmonary blastoma (PPB). Methods: Selected 3 PPB cases collected from the Hunan Children’s Hospital in a period from March 2001 to March 2015 (corresponding to sample 1, 2 and 3 in this paper, respectively) and 1 consultation recurrence case from the Beijing Children’s Hospital in 2015 (corresponding to sample 4). Subsequently, a comprehensive analysis and a prognosis tracking result were reported based on these 4 cases, including 2 girls and 2 boys, and the age ranged from 21 to 59 months. All the 4 cases originated from non-specific symptoms, such as cough and wheezy; and the imaging examinations showed pneumothorax, atelectasis or pulmonary cystic lesions. Results: All 4 cases were diagnosed as type Ⅱ PPB with the original embryonic compositions mixed with sarcomatoid regions, which can differentiate into rhabdomyoblasts or cartilage, under microscope. Immunohistochemically, Vimentin and Desmin were all expressed, Myogenin and CK-pan under benign epithelial expressed in 2 cases, and p53 in only 1 case. Sample 4 was diagnosed as type Ⅰ PPB after the first surgery, under the microscope rhabdomyoblasts and small round cells were distributed on the benign ciliated columnar epithelium, and the immunohistochemical test expressed CK-pan, Vimentin and Myogenin. Conclusion: PPB is rare in children and characterized by the original embryonic compositions mixed with sarcomatoid regions under microscope. As type Ⅰ PPB shows confusing features in both morphology and microscope, thus we need more biopsy tissue to avoid underestimates.