ASNS is a disease gene associated with asparagine synthetase deficiency (MIM: 615,574) and is a phenotype match for the proband, who presented with Lennox-Gastaut syndrome, infantile spasms, microcephaly, hypotonia, nystagmus, optic nerve hypoplasia, partial agenesis of the corpus callosum, and delayed myelination. This evidence concerns the gene ASNS and Congenital microcephaly-severe encephalopathy-progressive cerebral atrophy syndrome.