To analyze the clinical, imaging and pathological findings of congenital intrapulmonary lymphangioma and hemangioma in 5 infants and young children. Data of 3 cases with congenital intrapulmonary lymphangioma and 2 cases with haemangioma were analyzed. All the 5 cases had cough, difficulty in breathing, cyanosis of lips, and shortness of breath. Imaging study indicated cystic and space occupying changes of partial lung. In the two cases of hemangioma, the blood vessels passing through the hemangioma were observed on CT films. Histochemical studies showed that the cystic pockets from removed tissues were different in size and there was a line of flat endothelial tissue around these pockets. Immunochemical studies indicated D2-40 positive, factor VIII weak positive for lymphangioma cases; while in hemangioma cases, factor VIII was positive, D2-40 was negative or weakly positive, and both of cytokeratin was negative. It is very important to consider and identify congenital intrapulmonary lymphangioma or hemangioma when a patient has cystic pockets and space occupying change in their lung by imaging and pathological studies.