Pulmonary hypertension is a complex condition in which there is a significant increase in pressure in the pulmonary artery, contraction and remodeling of blood vessels, which leads to increased pulmonary vascular resistance, right ventricular hypertrophy and heart failure. The etiology of pulmonary hypertension is multifaceted and its common pathological basis is mitochondrial dysfunction. Mitochondrial dysfunction is closely associated with endothelial cell damage, which directly affects the metabolism and function of pulmonary vessels. The study identifies multiple pathogenic mechanisms, including mitochondrial DNA damage, electron transport chain dysfunction, protein homeostasis imbalance, mitochondrial biogenesis defects, dynamic abnormality, and autophagic defect. Current treatment approaches focus on relaxing the pulmonary vasculature, but their efficacy is limited because they do not reverse vascular remodeling. Mitochondrial dysfunction represents a promising direction for the development of new therapeutic strategies in the treatment of pulmonary hypertension. The study summarizes the latest knowledge about the molecular mechanisms of mitochondrial dysfunction and their role in the pathogenesis of pulmonary hypertension.