Pulmonary hypertension in children is a disorder associated with increased pulmonary vascular resistance and arterial pressure, decreased cardiac output, and right-sided cardiac dysfunction that is caused by numerous etiologies. Although treatment will vary with underlying cause, pharmacological treatment has historically included inhaled nitric oxide and prostacyclin analogues. Over the past several years new agents have been added to the treatment armamentarium, including phosphodiesterase V inhibitors (eg sildenafil) and endothelin antagonists (eg bosentan). Further, more agents are currently under investigation for pulmonary hypertension in children including immunosuppressives and other endothelin antagonist entities. Limitations to treatment include the availability of appropriate, robust pediatric pharmacological data and constraints with dosage forms. P ulmonary hypertension (PHN) in children is a disorder associated with increased pulmonary vascular resistance and arterial pressures, decreased cardiac output, and right-hand side cardiac dysfunction. In the 1970s, PHN carried a prognosis of less than 1 year of life following diagnosis. 1 However, with advancements in pharmacological treatment over the past three decades, the prognosis improved. Current estimates for the 5-year survival rate in pharmacologically-treated children are now as high as 90%. 1 Though once considered rare, PHN is now diagnosed at an increased frequency; however, the actual incidence of PHN in children still remains unknown. 2 Several classes of drug therapy exist for the treatment of various etiologies of PHN in neonates, infants and children. These agents include inhaled nitric oxide, prostacyclins, phosphodiesterase V inhibitors, endothelin antagonists, calcium channel blockers (CCBs), and several other novel therapeutic approaches. This review serves to introduce the reader to the existing data for pharmacological treatment and provide insight into therapeutic approaches to optimizing therapy of PHN and appropriate goals of therapy (Table 1).Pulmonary hypertension is caused by numerous etiologies and can be due to acute or chronic changes, and may be transient or permanent. Pulmonary hypertension is divided into 2 main categories, primary and secondary; primary PHN is generally idiopathic and sporadic, with a link to familial inheritance in up to 10% of cases. 2 Secondary PHN is associated with underlying disorders including, but not limited to, pulmonary arterial hypertension (associated with congenital heart disease and connective tissue diseases) or PHN associated with respiratory