Inhaled nitric oxide (INO) therapy is becoming an indispensable measure in some critically ill patients with pulmonary hypertension. Most importantly, it has been shown that INO significantly reduces the necessity for extracorporeal lung assist in newborns with hypoxemic respiratory failure and pulmonary hypertension. A large number of basic and clinical studies are making great steps in delineating its physiology, techniques, side effects, and clinical efficacy. This article reviews the current knowledge of INO therapy including a novel device of nitric oxide production system.