Abstract: Pulmonary hypertension (PH) is a progressively chronic disease mainly affecting the pulmonary vascular bed. The main pathological basis is endothelial dysfunction, extensive pulmonary artery constrictive abnormality and pulmonary vascular remodeling, which can eventually lead to right heart failure and even death. Recent studies have shown that the RhoA-ROCK pathway plays an important role in regulating cellular functions, including vascular smooth muscle cell contraction, proliferation and migration. It is also deeply involved in important pathophysiological processes such as vascular tone regulation, endothelial cell dysfunction, oxidative stress and inflammatory responses. All the above points are closely related to the pathogenesis of PH. Therefore, this paper mainly discusses the detailed mechanisms of the RhoA-ROCK pathway involved in the development of PH, as well as recent advances in PH-targeted interventions related to this pathway.