Additionally, we also reviewed the literature and summarized the phenotypes of previously reported patients with variants in the NARS2 gene.<h4>Results</h4>Here, we present the phenotypic and genetic features of 2 unrelated Iranian infants presented with neurodevelopmental delay, seizures, hearing impairment, feeding problems, elevated serum lactate levels in addition to subdural hematoma and cerebral parenchymal hemorrhage in the brain magnetic resonance imaging (MRI) of one of the patients. Here, NARS2 is linked to Neurodevelopmental delay.