Abstract: As an important enzyme of metabolic processes in human body, isocitrate dehydrogenase (IDH) is involved in the three carboxylic acid cycle and catalysis of isocitric acid to α-ketoglutarate. IDH mutations lead to the accumulation of (R)-2-hydroxyglutarate which is not a normal metabolite in the body. High level 2-HG is closely associated with the occurrence of leukemia and other tumors. Inhibitors of IDH mutants can reduce the level of 2-HG, and therefore have become a hot spot in the development of cancer drugs. Herein, we reviewed the role of IDH mutation in tumors and the advances in the research of mutant IDH inhibitors.