Conventional treatment of pulmonary arterial hypertension (PAH) includes oxygen supplementation, calcium channel blockers, anticoagulation, digoxin, and diuretics. Calcium channel blockers have little or no effect on the majority of patients with moderate or severe PAH. Apart from the intravenous prostacyclin which remains the gold standard treatment for this life-threatening entity, newer drugs such as prostacyclin analogues, endothelin-receptor antagonists, and phosphodiesterase-5 inhibitors alone or in combination administered by means of different routes have been shown to improve oxygenation, hemodynamics, exercise tolerance, symptoms, and quality of life. Based on current WHO classification there are 5 groups of pulmonary hypertension. In this article, PAH is used exclusively for Group 1 of WHO classification. There are currently six FDA approved therapies for PAH. However, all these therapeutic agents and approaches have not offered yet a cure of PAH. This review article presents the recent advances in the management of PAH.