Familial chylomicronemia syndrome (FCS) is a congenital, severe form of hypertriglyceridemia, caused by mutations of the lipoprotein lipase (LPL) gene.[1] LPL catalyzes lipolysis in triglyceride-rich lipoproteins such as chylomicrons. The gene discussed is LPL; the disease is familial chylomicronemia syndrome.