Lipoprotein(a) [Lp(a)] is a genetically determined risk factor for atherosclerotic vascular disease. Several studies have described a correlation between high Lp(a) plasma levels and coronary heart disease, stroke, and peripheral atherosclerosis. In healthy individuals Lp(a) plasma concentrations are almost exclusively controlled by the apolipoprotein(a) [apo(a)] gene locus on chromosome 6q2.6-q2.7. More than 30 alleles at this highly polymorphic gene locus determine a size polymorphism of apo(a). There exists an inverse correlation between the size (molecular weight) of apo(a) isoforms and Lp(a) plasma concentrations. Average Lp(a) levels are high in individuals with low molecular weight isoforms and low in those with high molecular weight isoforms. Mean Lp(a) plasma levels are elevated over controls in patients with renal disease. Patients with nephrotic syndrome exhibit excessively high Lp(a) plasma concentrations, which can be reduced with antiproteinuric treatment. The mechanism underlying this elevation is unclear, but the general increase in protein synthesis caused by the liver due to high urinary protein loss is a likely explanation. Patients with end-stage renal disease (ESRD) also have elevated Lp(a) levels. These are even higher in patients treated by continuous ambulatory peritoneal dialysis than in those receiving hemodialysis. Lipoprotein(a) concentrations decrease to values observed in controls matched for apo(a) type following renal transplantation. This clearly demonstrates the nongenetic origin of Lp(a) elevation in ESRD. Both the increase in ESRD and the decrease following renal transplantation are apo(a) phenotype dependent. Only patients with high molecular weight phenotypes show the described changes in Lp(a) levels. In patients with low molecular weight types the Lp(a) concentrations remain unchanged during both phases of renal disease. As in the general population, Lp(a) is a risk factor for cardiovascular events in ESRD patients. In this patient group the apo(a) phenotype seems to be equally or better predictive of the degree of atherosclerosis than is Lp(a) concentration. Further prospective studies will be necessary to confirm these observations. Whether Lp(a) also plays a key role in the pathogenesis and progression of renal diseases needs further study. Controversial data on the role of the kidney in Lp(a) metabolism result from insufficient sample sizes of several studies. Due to the broad range and skewed distribution of Lp(a) plasma concentrations, large study groups must be investigated to obtain reliable results.