Objective: To explore the clinical, imaging, pathological feature and prognosis of congenital pulmonary airway malformation (CPAM). Methods: Retrospect analysis the clinical pathologic features of 73 cases of CPAM and review related literatures. Results: Patients’ age ranges from 1 day to 15 years, with mean age 4.3 years old. CPAM has no sex predilection. Histologic examination showed that 49 cases were classified as Stocker type I, 22 cases as type II, 2 cases as type III. All were unilateral lesion and involvement on the left side of the 29 cases, on the right side of 44 cases. Of the 73 cases studied, the associated malformations included cardiac anomalies(6 cases), arachnoid cyst (2 cases), invisible spinal bifida and thyroid dysplasia (1 case), brain development backwardness and hydrocephalus (1 case), wilm’s tumor (1 case). All of the 73 cases performed X-ray or CT examination: except mass-like change for 2 cases, the others showed single or multiple large air cystic lesions; honeycomb-like small cystic lesions, the cyst lack of lung marking. All cases were underwent lobectomy, Follow-up time ranges from 2~25 months, except 2 cases died of type III, the rest of 71 cases recovered well. Conclusion: CPAM is a relatively rare pulmonary lesion of congenital cystic adenomatoid malformation. Imaging analysis is a valuable tool to suggest CPAM, while definite diagnosis requires pathologic examination. Surgical excision, commonly lobectomy is recommended.