A homozygous missense pathogenic variant (c.1100C > T, p.Thr367Ile; NM_001167734.1) in VARS2, the gene encoding the mitochondrial valyl tRNA‐synthetase, was described in a patient with a clinical picture characterized by psychomotor delay and epilepsia partialis continua associated with a mitochondrial Complex I defect [Diodato et al., 2014ba,b]. Here, VARS2 is linked to epilepsia partialis continua.