Pulmonary hypertension (PH) is a fatal disease characterized by pulmonary vascular remodeling, ultimately leading to right heart failure and death. Current treatments for PH are suboptimal, with no substantial improvement in overall survival among patients with advanced PH. Despite some progress in understanding the pathogenesis of PH, further studies at the molecular level are needed to develop more effective treatments for PH. Recent research has shown that long non-coding RNAs (lncRNAs) have an important regulatory function in the pathophysiological process of PH, and may thus be potential disease biomarkers and therapeutic targets. In this paper, we review recent progress in our understanding of the molecular mechanisms of lncRNAs in PH.