In light of the evidence of oxidative stress in the VLCAD-deficient patient fibroblasts, we evaluated the level of nuclear factor (erythroid-derived 2)-like 2 (Nrf2) and nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB)—two important transcription factors involved in redox homeostasis and control of inflammation. This evidence concerns the gene NFKB1 and very long chain acyl-CoA dehydrogenase deficiency.