LIPA and cholesteryl ester storage disease: Lysosomal acid lipase deficiency (LAL-D) is a rare autosomal recessive genetic disorder associated with mutations in the gene LIPA, that encodes the enzyme lysosomal acid lipase (LAL).[1] Its clinical spectrum ranges from an earlier-onset presentation (Wolman ́s disease), with high mortality in the first 2 years of life, to a later-onset form, known as cholesteryl esters storage disease (CESD), which is characterized by a systemic accumulation of cholesteryl esters and triglycerides, including in hepatocytes and Kupffer cells.