Lipoprotein(a) (Lp(a)) is an increasingly discussed and studied risk factor for atherosclerotic cardiovascular disease and aortic valve stenosis. Many genetic and epidemiological studies support the important causal role that Lp(a) plays in the incidence of cardiovascular disease. Although dependent upon the threshold and unit of measurement of Lp(a), most estimates suggest between 20 and 30% of the world's population have elevated serum levels of Lp(a). Lp(a) levels are predominantly mediated by genetics and are not significantly modified by lifestyle interventions. Efforts are ongoing to develop effective pharmacotherapies to lower Lp(a) and to determine if lowering Lp(a) with these medications ultimately decreases the incidence of adverse cardiovascular events. In this review, the genetics and pathophysiological properties of Lp(a) will be discussed as well as the epidemiological data demonstrating its impact on the incidence of cardiovascular disease. Recommendations for screening and how to currently approach patients with elevated Lp(a) are also noted. Finally, the spectrum of pharmacotherapies under development for Lp(a) lowering is detailed.