After the finding of isocitrate dehydrogenase (IDH) 1/2 mutations6, 7, gliomas harboring mutant IDH and wild-type IDH have been considered to be distinct tumors that derived from different linkages because these two genetic subtypes have totally different characteristics including survival time, mean age at diagnosis, and tumor location8–10. The gene discussed is IDH3A; the disease is central nervous system cancer.