OBJECTIVE: The number of patients with end-stage renal disease has increased during the last decades. Data shows that 10% of the renal replacement therapy population in the intensive care unit are patients with end-stage renal disease. We aimed to describe the short- and long-term outcome of these patients after renal replacement therapy in the intensive care unit. DESIGN: Nationwide cohort study between the years 1995 and 2004. Follow-up up to 5 years. SETTING: Swedish general intensive care units and Swedish hospitals. PATIENTS: Eligible subjects were end-stage renal disease patients treated with renal replacement therapy in 32 Swedish general intensive care units. In total, 245 patients were studied. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Short- and long-term mortality was studied. Logistic regression was used to analyze short-term mortality. Long-term mortality was compared with the mortality of end-stage renal disease patients outside the intensive care unit and the mortality in the population. Diabetes and heart failure are significant risk factors for 90-day mortality, with an odds ratio of 1.9 and 2.0, respectively. The intensive care unit end-stage renal disease cohort had increased long-term mortality as compared with non-intensive care unit end-stage renal disease patients, relative risk of death 2.32 (confidence interval 1.84-2.92). A comparison with the mortality rate in the general population yielded a standardized mortality ratio of 25 (95% confidence interval: 19.6-31.4). CONCLUSIONS: For end-stage renal disease patients in the intensive care unit, age, diabetes mellitus, and heart failure are risk factors for 90-day mortality. Long-term mortality is associated with age and heart failure. The long-term mortality of end-stage renal disease patients surviving the intensive care unit stay is significantly higher compared with end-stage renal disease patients without a known intensive care unit admission.