Following the discovery of the Lipoprotein(a) (Lp(a)) molecule by Kare Berg in 1963, many physiological and pathological properties of this particle remain to be fully understood. Multiple population-based studies have demonstrated a correlation between elevated Lp(a) levels and the incidence of cardiovascular disease. Data extrapolated from the Copenhagen City Heart and ASTRONOMER studies also demonstrated the link between Lp(a) levels and the incidence and rate of progression of calcific aortic stenosis. Interest in Lp(a) has increased in recent years, partly due to new emerging therapies that can specifically reduce serum Lp(a) concentrations. Given the strong correlation between Lp(a) and CV disease from epidemiological studies, several international guidelines have also been updated to advocate Lp(a) testing in specific population groups. This review aims to highlight the importance of the role of Lp(a) in cardiovascular disease and discusses the potential of novel therapies in patients with elevated Lp(a) levels.