Abstract: Pulmonary vascular remodeling is the core pathological feature in the onset and progression of pulmonary arterial hypertension (PAH). Currently, there is no well-defined therapeutic strategy that can effectively delay or reverse this process. Despite the widespread clinical application of targeted vasodilator drugs, patients still face a high risk of mortality and adverse cardiovascular events, suggesting an urgent need to explore new pathological mechanisms and therapeutic targets. In recent years, the relationship between dyslipidemia and PAH has garnered increasing attention. This article aimed to review the role of lipid metabolism disorders in pulmonary vascular remodeling in pulmonary arterial hypertension and its underlying mechanism, with the hope of providing new intervention targets for the treatment of PAH, thereby improving patient survival rates and quality of life.