In summary, this report highlights three key novelties: (1) the first documented case of cerebral infarction in NARS2-related COXPD24, expanding the neuroimaging and clinical phenotype; (2) identification of a large exon 6–11 deletion and a novel missense variant, enriching the mutational spectrum and emphasizing the need for CNV analysis; and (3) mechanistic insights suggesting that mitochondrial energy failure may precipitate focal metabolic stroke-like lesions, providing a conceptual framework for understanding acute neurovascular complications in mt-aaRS–related disorders. Here, NARS2 is linked to brain infarction.