The most typical clinical presentations are leukoencephalopathy with brainstem and spinal cord involvement and high lactate (LBSL) due to DARS2 mutations 8, leukoencephalopathy with thalamus and brainstem involvement and high lactate (LTBL) caused by EARS2 mutations 9, but other mt‐ARS mutations may also cause white matter lesions. Here, EARS2 is linked to Leukoencephalopathy.