Abstract: Gliomas are the most frequent and lethal cancers of the central nervous system (CNS). Despite recent therapeutic advances in surgical resection, adjuvant radiation therapy, and chemotherapy, the results remain unsatisfactory. Glioma grades increase and become more malignant, inducing patient morbidity and mortality. With the increasing knowledge regarding the glioma genetic aberrations, mutations of the gene encoding isocitrate dehydrogenase-1 (IDH1) have been shown in a significant glioma proportion, usually producing a good outcome. IDH1 mutations are useful genetic alterations for the diagnosis, classification, novel targeting, and good prognosis of gliomas. A comprehensive review on the role of IDH1 mutation in gliomas is provided in this study.