There is mounting interest in the concept of endothelial progenitor cell (EPC) therapy for the treatment of pulmonary arterial hypertension (PAH). Recent successful pilot studies in idiopathic PAH have raised questions about the contribution of progenitor cells circulating in the peripheral blood to pulmonary vascular homeostasis and to the process of vascular remodelling. This review will summarise the work performed to date in animal and human therapeutic trials and clarify what is known about the potential contribution of EPCs to the pathophysiology of PAH.