The aim of the study was to investigate the risk of retinal vein occlusion (RVO) following end-stage renal disease (ESRD). The study was designed as a retrospective, nationwide, matched cohort study. The subjects were ESRD patients identified by the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM), code 585. The study cohort included 92,774 ESRD patients registered between January 2000 and December 2009 at the Taiwan National Health Insurance Research Database. An age- and sex-matched control group comprised 92,774 patients (case:control = 1:1) selected from the Taiwan Longitudinal Health Insurance Database 2000. Information for each patient was collected from the index date until December 2011. The incidence and risk of RVO were compared between the ESRD and control groups. The adjusted hazard ratio (HR) for RVO after adjustment for potential confounders was obtained by Cox proportional hazard regression analysis. Kaplan-Meier analysis was used to calculate the RVO cumulative incidence rate. The main outcome measure was the incidence of RVO following ESRD. In total, 904 ESRD patients (0.97%) and 410 controls (0.44%) had RVO (P < 0.0001) during the follow-up period, leading to a significantly elevated risk of RVO in the ESRD patients compared with controls (incidence rate ratio = 3.05, 95% confidence interval = 2.72-3.43). After adjustment for potential confounders including diabetes mellitus, hypertension, hyperlipidemia, congestive heart failure, and coronary artery disease, ESRD patients were 3.05 times more likely to develop RVO in the full cohort (adjusted hazard ratio = 3.05, 95% confidence interval = 2.64-3.51). In addition, hypertension patients showed high incidence rate of RVO in the ESRD group compared with controls (incidence rate ratio = 1.71, 95% confidence interval = 1.44-2.03) and maintained significant risk of RVO after adjustment for other confounders in the cohort (adjusted hazard ratio = 1.39, 95% confidence interval = 1.20-1.60). ESRD increases the risk of RVO. For ESRD patients, we recommend education regarding RVO in addition to blood pressure control to prevent subsequent RVO.