We report a case of a 42-year-old male with chronic thromboembolic pulmonary hypertension. His preoperative examination revealed severe hypoxemia (PaO2 48 mmHg, PaCO2 34 mmHg in room air), a mass in the right ventricle and severe pulmonary hypertension (pulmonary arterial pressure 70/33 mmHg). We successfully performed right ventricular thrombectomy to prevent further embolization from the right ventricular thrombus. Using inhaled low dose nitric oxide (NO) during perioperative period, weaning from cardiopulmonary bypass and ventilator were easily done. In this case, inhaled NO was successfully administered for the perioperative management of chronic pulmonary hypertension.