The authors report a case of congenital cystic adenomatoid malformation of the lung (CCAM) with extramedullary hematopoiesis (EMH). A baby girl suffered from Rh incompatibility and hemolytic anemia. She exhibited respiratory distress. Chest radiography and magnetic resonance imaging findings showed a CCAM. A resection of the upper and middle lobes of the right lung was performed. Histological examination showed a CCAM of Stocker's type II with EMH. EMH in the lung is very rare. The authors speculate that the cause of our case originated from Rh incompatibility and development arrest in the CCAM lung.