End-stage renal disease (ESRD) attributed to renovascular disease (RVD-ESRD) has been incompletely characterized. We determined incidence trends, clinical features, prior treatment, and survival of patients with RVD-ESRD using the US Renal Data System database. Primary causes of ESRD were assessed in patients starting ESRD therapy during 1991 to 1997. The incidence of RVD-ESRD increased from 2.9/10(6) per year (1.4% of new ESRD cases) to 6.1/10(6) per year (2.1%). The annualized increase was 12.4% per year. This is a greater rate of increase than for ESRD from diabetes mellitis (DM-ESRD; 8.3% per year) and ESRD overall (5.4% per year). The risk for RVD-ESRD versus other-cause ESRD correlated positively with age (odds ratio [OR], 1.7 per 10-year increment; P < 0.0001) and male sex (OR, 1.2; P < 0.0001) and negatively with black (OR, 0.17; P < 0.0001), Asian (OR, 0.29; P < 0.0001), and Native American race (OR, 0.31; P < 0.0001). The unadjusted prevalence of coronary heart disease, cerebrovascular disease, and peripheral vascular disease was greater in patients with RVD-ESRD versus other-cause ESRD (P < 0.001). Of patients with RVD-ESRD, 5% underwent revascularization in the 2 years before ESRD compared with 0.5% of patients with other-cause ESRD, including DM-ESRD. Adjusted for age, race, sex, comorbidity, and laboratory values, the survival of patients with RVD-ESRD was similar to that for patients with other-cause ESRD (risk ratio, 1.01; P = 0.5). These findings suggest that RVD-ESRD is increasing faster than other-cause ESRD and is not independently associated with an increased mortality risk. Strategies may exist to prevent progression to ESRD and merit priority for further study.