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South Asian individuals (SA), representing approximately one quarter of the global population, experience a disproportionately high burden of cardiovascular disease. Some of this increased susceptibility is accounted for by traditional risk factors such as diabetes, hypertension, carbohydrate-rich diets, and rising rates of obesity and metabolic syndrome. However, other previously underappreciated risk factors may also play a crucial role. These include environmental pollution, genetic factors, and Lp(a) (lipoprotein(a)). Various epidemiological and genetic studies support the role of Lp(a) as a causal and independent risk factor for atherosclerotic cardiovascular disease. SA have a higher prevalence of elevated Lp(a) levels (25% have levels >50 mg/dL) compared with Western populations, and this may be one factor that accounts for the earlier age of onset of coronary artery disease, its more aggressive course, and higher morbidity and mortality in this group. SA experience myocardial infarction nearly 10 years earlier than individuals of European descent and have higher rates of premature and multivessel coronary artery disease. Additionally, socioeconomic shifts, cultural practices, and disparities in health care access may further exacerbate these risks, creating a complex interplay of factors that heighten cardiovascular vulnerability in SA. In this article, we review the data on the role of Lp(a) in mediating atherosclerotic cardiovascular disease, its epidemiology in SA, current screening guidelines, and drugs in the pipeline that will potentially be able to reduce high Lp(a) levels and associated cardiovascular risk. Ultimately, outcome trials with such drugs will be needed in this large population to examine their efficacy and safety.