Hallmark laboratory abnormalities include high LDL, total cholesterol, and triglycerides which are mainly attributed to activation of de novo lipogenesis via SREBP1c as a result of low cytoplasmatic concentration of free cholesterol and fatty acids.2 Low HDL is a consequence of decreased activity of LXR-dependent HDL production.2 Children or adults with LAL-D usually progress to end-stage liver disease with its complications early in life and are at high risk of developing premature atherosclerosis with cerebral or myocardial infarction. This evidence concerns the gene SREBF1 and myocardial infarction.