The NM_000018.4(ACADVL): c.480C>G (p.Tyr160Ter) variant in ACADVL is a nonsense variant predicted to cause a premature stop codon in biologically-relevant-exon 7/20 leading to nonsense mediated decay in a gene in which loss-of-function is an established disease mechanism (PVS1; PMIDs 9973285, 11590124). This variant is absent from gnomAD v2.1.1 (PM2_Supporting). To our knowledge, this variant has not been reported in the literature either in a patient with very long chain acyl-CoA dehydrogenase (VLCAD) deficiency or ACADVL associated disease or in functional studies. In summary, this variant meets the criteria to be classified as likely pathogenic for autosomal recessive VLCAD deficiency based on the ACMG/AMP criteria applied, as specified by the ClinGen ACADVL Variant Curation Expert Panel: PVS1, PM2_supporting (ACADVL VCEP specifications version 1; approved November 9, 2021). The ACADVL Variant Curation Expert Panel VCEP classified the variant as likely pathogenic based on PVS1+PM2_supporting. This variant was originally curated November 29, 2021 and the recurated classification was approved by the expert panel on February 27, 2024.