Congenital cystic adenomatoid malformation (CCAM) is a rare disease that is diagnosed by severe respiratory dysfunction from birth. A 5-day-old-boy with CCAM underwent removal of a large cyst which was present at lower lobe of right lung. Anesthesia was induced slowly and maintained with oxygen and sevoflurane. Severe airway obstruction occurred transiently by the secretion from the lung cyst. Thereafter, the surgery was completed safely and his postoperative course was uneventful. Perioperative anesthetic management of the patient with CCAM is also discussed.