A 1-month-old male infant with respiratory distress was referred to our hospital for operation of the ventricular septal defect. A chest roentgenogram demonstrated pulmonary emphysema especially in the right upper and middle lobes. At 3 months, a perimembranous ventricular septal defect was closed. But the infant could not be weaned from the ventilator. On the 21st postoperative day, a right upper and middle bilobectomy was performed. Three days later, he was weaned from the ventilator and the postoperative course was uneventful. The pathologic diagnosis revealed no bronchial cartilagenous abnormality. Infantile lobar emphysema of the right upper lobe with congenital heart disease is rather rare. We emphasize the need for lobectomy simultaneous with, secondary to, cardiac surgery in these cases.