Pulmonary arterial hypertension (PAH) is a rare form of pulmonary hypertension characterized by a progressive obliterative vasculopathy of the distal pulmonary arterial circulation that usually leads to right ventricular failure and death. Over the last 25 years, more than a dozen drugs representing five drug classes have been developed and approved for the treatment of this devastating disease. Due to the small number of patients afflicted by PAH, most health care providers have little experience with its management. To address this gap in medical knowledge, treatment guidelines have been developed by professional organizations and expert committees. Over the last few years, these guidelines have been updated to address findings from recent clinical trials and ongoing experience with these drugs. This review provides an update on the most recently published treatment guidelines for pharmacologic treatment of PAH and incorporates them into a contemporary approach to the treatment of this disease.