The 2016 World Health Organization (WHO) classification of tumors of the central nervous system included well-established molecular signatures, such as isocitrate dehydrogenase (IDH) mutation status, expression of the transcription regulator ATRX, and 1p/19q codeletion status [2], where IDH is a small molecule protein involved in a number of cellular processes, including mitochondrial oxidative phosphorylation, glutamine metabolism, lipogenesis, glucose sensing, and regulation of cellular redox status [3–5]. Here, IDH3A is linked to central nervous system cancer.