The administration of NO has become an important and effective therapy in the clinical management of pulmonary hypertension associated with cardiopulmonary disorders in infants and children. It is likely to become a routine therapy in the treatment of PPHN, although dosing and timing strategies, early indicators of treatment failure, and long-term outcomes are not completely understood. The use of NO has also been beneficial in the evaluation and management of pulmonary hypertension associated with congenital heart disease. The role of NO in the management of pediatric ARDS holds promise, although further clinical trials are needed. Additional research should also be directed toward the use of NO in preterm infants and those born with CDH. Future endeavors may also include the use of NO in the evaluation and management of asthma.