Pulmonary arterial hypertension (PAH) is a progressive, life-threatening condition. Sitaxsentan, a selective endothelin-A receptor antagonist, is an effective, safe and well-tolerated endothelin receptor antagonist for the treatment of PAH in adult patients. Multi-center, randomized, placebo-controlled clinical trials have demonstrated that sitaxsentan has beneficial effects on exercise capacity (i.e., 6-min walk distance), functional class and hemodynamic parameters in PAH patients. Sitaxsentan has a low incidence of acute hepatotoxicity. Patients on concomitant warfarin require a decrease in warfarin dose to maintain a therapeutic international normalized ratio. The demonstration of clinical efficacy and low incidence of acute hepatotoxicity support the potential use of sitaxsentan for the treatment of PAH.