Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare pulmonary malformation, associated with local aberrations of parenchymal tissue development, which have been classified lung bud anomalies. Lung bud anomalies include infantile lobar emphysema, congenital cyst of the lung, congenital cystic adenomatoid malformation, pulmonary sequestration and bronchogenic cyst. CCAM can enlarge rapidly by ball-valve air entrapment by cysts, lead to mediastinal shift and compression of the heart. compression of opposite lung may result in pulmonary hypoplasia and CCAM causes acute respiratory distress in newborn. We report a neonate with CCAM who were successfuly managed during perioperative period.