Hypoxic pulmonary hypertension (HPH) is a refractory disease. The main symptoms of HPH are pulmonary vascular remodeling and persistent increase in pulmonary vascular resistance, which can lead to progressive right ventricular hypertrophy and even death. The role of platelet-derived growth factor-BB/ERK/hypoxia-inducible factor-1α signaling pathway in the occurrence and development of hypoxic pulmonary hypertension has become the focus of attention. The hyperbaric treatment provides new ideas. This review mainly studies the research progress of the PDGF-BB/ERK/HIF-1α signaling pathway in pulmonary hypertension in recent years and conducts related research.