Pulmonary hypertension is an elevation in pulmonary artery pressure that is associated with a spectrum of diseases and causes. Its clinical severity and presentation are widely varied. The field of study has changed immensely over the past several years. Significant knowledge has been gained in the pathophysiology, genetics, and vascular biology associated with pulmonary hypertension. These discoveries have contributed to medical interventions that have improved outcomes associated with pulmonary hypertension. This article reviews pulmonary hypertension in children, focusing on idiopathic pulmonary hypertension. Because most information is associated with children who have this form of the disease, formerly classified as primary pulmonary hypertension, medical therapy is discussed with a focus on this patient group. Additional therapeutic concepts relevant to other causes of pulmonary hypertension are highlighted.