Pulmonary arterial hypertension (PAH) is a rare, but serious condition which, if untreated, is associated with a poor survival. Currently, even if several trials have led to the approval of many drugs for PAH, there is no established cure for this disease. However, approved drugs for PAH have contributed to significantly improve symptoms, exercise capacity, quality of life and survival of these patients. The aim of this review is to overview the standard treatment of PAH and to give some insights about new treatments that are currently under investigation along with the discussion of recent patents.