The aim of this literature review is to present the role of IDH1 and IDH2 mutations in the pathogenesis of AML and the results of clinical trials using mutant IDH1 (mIDH1) and mutant IDH2 (mIDH2) inhibitors in newly diagnosed and R/R AML, and to discuss the challenges related to the use of mIDH1 and mIDH2 inhibitors in practice (the mechanisms behind the development of resistance to treatment and the side effects of the drugs) and future prospects related to the potential methods of overcoming resistance to treatment with mIDH1 and mIDH2 inhibitors. The gene discussed is IDH1; the disease is acute myeloid leukemia.