Pulmonary lymphangioleiomyomatosis (PLAM) is a rare chronic multi-system neoplastic disease that occurs in women of childbearing age. It lacks specific clinical manifestations and requires reliable biomarkers to achieve precise management. In recent years, with the emergence of emerging biomarkers, the detection rate of PLAM has been significantly improved, which can better monitor disease progression and provide timely feedback on the efficacy. These emerging biomarkers mainly include vascular endothelial growth factor-D, vitamin D-binding protein, CT score and prostaglandins. This article will focus on the current research results, and summarize the research progress of emerging biomarkers in PLAM diagnosis, prognosis evaluation, and disease monitoring, aiming to provide new ideas for the research and treatment of PLAM.