Biallelic variants in MRPS2 have been recently reported to cause combined oxidative phosphorylation deficiency 36 (COXPD36, MIM# 617950), presenting with variable phenotypes of global developmental delay, ataxia, hypotonia, severe lactic acidosis, hypoglycemia, and sensorineural hearing loss in four unrelated individuals till date [8–10]. The gene discussed is MRPS2; the disease is cerebellar ataxia.