More than 15 distinct disorders have been described as affecting FAO; These include glutaric aciduria type 2, primary carnitine deficiency and deficiencies of carnitine palmitoyltransferase 1A (CPT1A), carnitine acylcarnitine translocase (CACT), very long chain acyl-CoA dehydrogenase (VLCAD), long chain hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein (LCHAD/MTP), medium chain acyl-CoA dehydrogenase (MCAD), medium/short chain hydroxyacyl-CoA dehydrogenase (M/SCHAD), and short chain acyl-CoA dehydrogenase (SCAD) [2–4]. Here, ACADL is linked to systemic primary carnitine deficiency disease.