Abstract: Isocitrate dehydrogenase (IDH) mutation occurs frequently in glioma, cholangiocarcinoma, acute myeloid leukemia, chondro sarcoma and other tumors, and it's a potential target for cancer treatment. The novel cancer metabolite 2-hydroxyglutaric acid (2-HG) produced after mutation has become an important marker for cancer treatment and prognosis evaluation. IDH mutation accurately down-regulates various biological processes such as glucose metabolism, lipid metabolism and amino acid metabolism in tumors. In addition, IDH mutation also affects the balance of immune microenvironment and epigenetic regulation, and its mechanism of action in the occurrence and development of cancer needs to be further explored and studied. This article reviews the mechanism of action and potential therapeutic strategies of IDH mutation in the development of tumors, aiming to provide novel therapeutic options for patients with IDH mutation.