Pulmonary arterial hypertension (PAH) is a group of severe and progressive diseases, and eventually leading to right heart failure even death. Pulmonary vasoconstriction, Pulmonary vascular remodeling , thrombosis, and vascular stiffness are the major causes for PAH. Numerous studies have shown that Ca2+ signal plays indispensable role in maintaining vascular tension and the regulation of vascular smooth muscle cell proliferation and apoptosis, and these processes are mainly mediated by calcium channels. This paper will focus on the advances of calcium channels in the pathogenesis of pulmonary arterial hypertension.