Molecular structure and cellular distribution of lymphocyte activation gene-3 (LAG-3) have been studied extensively since 1990. However, several unresolved questions remain. It is well-established that LAG-3 plays a significant role in maintaining immune homeostasis. The presence of deficiencies in LAG-3 has been observed to be linked with autoimmune disorders, whereas the excessive expression of LAG-3 within the tumor microenvironment hinders immune responses, particularly those mediated by lymphocytes, thereby facilitating immune evasion. Consequently, investigations into these 2 aspects have become a prominent focus in both fundamental and clinical research. The objective of this review is to examine the functions and molecular characteristics of LAG-3, as well as its current clinical applications in the context of tumor immune escape and autoimmune disease. The ultimate aim is to explore and propose novel immune therapy approach.