Congenital lobar emphysema (CLE) is a rare entity of unknown incidence. The main signs and symptoms are tachypnea, tachycardia, cyanosis, retractions, wheezing, tympanic chest percussion, asymetric breath sounds, or displaced cardiac tones within the first month of life. Occasionally a superimposed pulmonary infection exacerbates the condition, prompting hospital admission and medical treatment of the pneumonia followed by surgical treatment of the CLE. We now report a case of left upper CLE in a 15-day-old infant. Anesthesia was induced with thiopental sodium and maintained with intravenous fentanyl and midazolam. Endotracheal intubation was uneventful. Muscle relaxation was done with rocuronium. Gentle manual ventilation with a Mapleson D circuit was begun and changed to intermittent mandatory ventilation with an infant ventilator. During the left upper lobe resection, we used high frequency oscillation ventilation (HFOV) at FIO2 1.0, 12 Hz frequency, 12 cmH2O amplitude for 25 minutes. The minimal lung movement during HFOV was found to provide excellent operating conditions for the surgeons and adequate oxygenation without cardiovascular compromise. The PaCO2 was increased to 71 mmHg 25 minutes after the start of HFOV, and returned to normal value with intermittent mandatory ventilation. The remainder of the operation and anesthesia were uneventful. The patient was transferred to the neonatal intensive care unit.