Lysosomal acid lipase deficiency (LAL-D) is a lysosomal storage disorder caused by mutations in the lipase A (LIPA) gene that result in a failure of the LAL enzyme to sufficiently hydrolyze cholesteryl esters and triglycerides into free cholesterol and free fatty acids in the lysosome.1, 2, 3, 4 LAL occupies a critical and essential position in the control of plasma lipoprotein levels and in the prevention of cellular lipid overload, especially in the liver and spleen.2 This evidence concerns the gene LIPA and lysosomal storage disease.