The establishment of neomorphic activity associated with the IDH mutation has long been shown to have an effect on cellular metabolism, cancer biology and oncogenesis.16,17 In this review, we will outline the biological impact of IDH-mutant neomorphic activity on cellular metabolism, epigenetic regulation and redox homoeostasis and address key advances in the cancer biology of IDH-mutated glioma and the implication of these findings in the development of future therapeutics, with a particular focus on glioma. The gene discussed is IDH1; the disease is cancer.