Despite reporting of a strict genotype–phenotype association for many mt‐aaRS, in some cases mutations in the same gene have been associated with very different clinical phenotypes; for example, pathogenic variants in AARS2 (encoding for alanyl‐tRNA synthetase) are associated either with infantile mitochondrial cardiomyopathy [Götz et al., 2011] or leukodystrophy with ovarian failure [Dallabona et al., 2014]. This evidence concerns the gene AARS2 and ovarian dysfunction.