Background: This systematic review provides an overview of the extant literature on progressive resistance training (PRT) in patients with end-stage renal disease (ESRD) and outlines recommendations for future trials. Methods: A systematic review of all published literature evaluating the chronic (>6 weeks) application of PRT in patients with ESRD using electronic databases. Results: The search yielded 16 clinical trials, including 11 randomized controlled trials (RCT), 4 uncontrolled trials and one trial involving a within-subjects control period plus RCT. RCT quality, assessed via the CONSORT statement, ranged from low (4/10) to high (10/10) with a mean score of 7.3/10; 7/11 RCT had a quality score ≥7.5. All trials evaluated chronic adaptation to PRT across a range of important outcomes. PRT can induce muscle hypertrophy and improve aspects of physical functioning and health-related quality of life in ESRD. There is preliminary evidence that PRT may reduce protein-energy malnutrition and cardiovascular disease risk factors, including C-reactive protein, total cholesterol, triglyceride, and measures of insulin resistance in patients with or at-risk of comorbid type 2 diabetes. The evidence base for PRT adapting some of the endpoints investigated to date remains inconsistent (e.g. physical performance tests, obesity outcomes), and many other pertinent clinical outcomes remain to be investigated. Conclusion: RCT are required to investigate a range of novel research questions related to the benefits and application of PRT in this cohort and its patient subgroups (e.g. diabetes, depression, dyslipidemia, etc.). Future studies must be of high methodological quality to inform clinical practice guidelines.