When LAL activity is strongly reduced or absent, due to homozygous or heterozygous mutations in the LIPA gene, cholesteryl esters and triglycerides accumulate prevalently within lysosomes, and several complications arise due to both lipid overload at the target organ level and to dyslipidaemia, secondary to upregulation of endogenous cholesterol production by hydroxyl-methyl-glutaryl coenzyme-a (HMG-CoA) reductase [4]. Here, LIPA is linked to inherited lipid metabolism disorder.