Pulmonary arterial hypertension is a chronic pulmonary vasculature disorder characterized by pulmonary vascular remodeling and elevated pulmonary artery pressures. Clinical manifestations, laboratory work-ups, cardiopulmonary exercise testing and 6-minute walk distance are helpful in reaching the correct diagnosis. The definite diagnosis relies on right heart catheterization. Pulmonary perfusion imaging with computed tomographic pulmonary angiography is similar to the effect of pulmonary angiography, showing important diagnostic values. Routine biochemistry, hematology and thyroid function tests as well as antinuclear antibodies can be helpful. Conventional medication, palliative septostomy and lung transplantation are treatments of choice. Novel techniques including microRNA, mesenchymal stem cell and remote postconditioning treatments have achieved initial therapeutic effects. Recent studies have demonstrated the potential effectiveness of inhaled nitric oxide, milrinone, phosphodiesterase-5 inhibitors and prostacyclin analogues for the treatment of PAH in pediatric patients.