Pulmonary hypertension (PH) is a debilitating progressive disease characterized by increased pulmonary arterial pressures, leading to right ventricular (RV) failure, heart failure and, eventually, death. PH is a rare disease involving many disciplines and has different epidemiological characteristics in different parts of the world. There are many causes of PH, but its pathogenesis has not been fully elucidated. The occurrence and development of PH is closely related to pulmonary vascular remodeling and abnormal function. At present, the treatment of PH includes drug treatment and non-drug treatment. These treatments can effectively improve the symptoms of PH patients, prolong the life of patients, but can’t completely cure the symptoms of PH, therefore, in order to find some more effective treatments, people need to invest more time and energy to study its pathogenesis. Exosomes have gradually become the focus of research, exosomes are rich in nucleic acids and proteins, including a large amount of microRNAs. Studies have shown that exosomes microRNAs from different cells or tissue sources play an important role in cardiovascular diseases including PH and is expected to become a new way to treat PH. Therefore, this article reviews the role of microRNAs from different cells or tissue sources in the occurrence and development of PH.