Premature infants with respiratory distress syndrome may have clinically significant shunting through a patent ductus arteriosus (PDA). Left-to-right shunting through the PDA may lead to left ventricular volume overload and pulmonary edema. We present a case of perioperative management for severe respiratory distress syndrome in a premature infant who underwent surgical closure of PDA. Under general anesthesia, the infant was successfully managed by inhaled nitric oxide, high frequency oscillation ventilation with intermittent mandatory ventilation despite intermittent hypoxia. The operation was performed safely in the neonatal intensive care unit.