Arachidonic acid (AA) is an all cis-5,8,11,14-eicosatetraenoic acid. A variety of arachidonic acid derivatives can be synthesized from AA through cyclooxygenase (COX), lipoxygenase (LOX) and cytochrome P450 (CYP) pathways and play an important role in pathological and physiological vascular remodeling. Pulmonary arterial hypertension (PAH) is a pathological process characterized by augmentation incessantly of pulmonary artery resistance and pulmonary artery pressure, which eventually leads to right heart failure and even death. Pulmonary vasoconstriction, vascular remodeling and vascular embolism are important pathological basis. In this paper, we focused on the role of arachidonic acid and its derivatives in the development of PAH and its underlying mechanism, which provide ideas for the discovery of new drug targets for the treatment of PAH.