Cumulative evidence suggests that lipoprotein(a) [Lp(a)] exerts an independent effect on the initiation and progression of atherosclerotic cardiovascular disease. The genetically mediated expression of apolipoprotein(a), which is the key structural and functional component of Lp(a), occurs in hepatocytes with subsequent extracellular Lp(a) assembly at the hepatic cell surface. Here we describe a case of elevated Lp(a) concentrations identified after (and likely acquired by) orthotopic liver transplantation, that contributed to accelerated atherosclerotic cardiovascular disease despite intensive therapeutic interventions. This case study represents an important example to include Lp(a) screening in routine lipid panel testing for all liver transplant donors and recipients; to reduce unanticipated and debilitating cardiovascular morbidity and mortality.