Chronic kidney disease is associated with a tremendously increased risk for cardiovascular disease. Traditional risk factors for cardiovascular disease, however, show a diminished predictive power in these patients compared with the general population. This review provides an overview of lipoprotein(a), which is considered a nontraditional risk factor. The characteristic genetic and nongenetic changes of lipoprotein(a) in kidney disease are discussed and set into the context of risk prediction. In particular, genetically determined apolipoprotein(a) polymorphism is a powerful risk predictor for cardiovascular disease and total mortality in these patients. Finally, the limited interventional strategies available to lower lipoprotein(a) are considered.