Secondary CoQ10 defect has also been described in non-mitochondrial disorders linked to genes such as APTX coding for aprataxin and causing ataxia occulomotor-apraxia [7], BRAF coding for serine/threonine-protein kinase B-Raf and causing cardiofaciocutaneous syndrome [10], ACADVL causing very long-chain Acyl-CoA dehydrogenase deficiency or NPC causing Niemann-Pick-type C disease [11]. Here, ACADVL is linked to long chain acyl-CoA dehydrogenase deficiency.