INTRODUCTION: Chronic kidney disease is associated with several negative factors that may counteract the potential effects of strength training. The purpose of this systematic review was to explore the effects of strength training on muscle mass, muscle strength, physical function and quality of life in patients undergoing dialysis. METHODS: A literature search was conducted in The Cochrane Library, MEDLINE, Embase, CINAHL and PEDro. Eight randomised, controlled trials of patients undergoing haemodialysis (n = 290) were included. RESULTS: There were inconsistencies in the results on the effects of strength training on muscle mass. Muscle strength was improved in six of eight tests. Objectively tested physical function remained unchanged. Pooled data for self-rated physical health and physical function from Short Form 36 were improved with strength training (mean (95% confidence interval) 10.05 (2.95-17.14), p = 0.006, and 9.38 (0.79-17.97), p = 0.03, respectively). CONCLUSIONS: The results suggest that it may be difficult to increase muscle mass with strength training in patients who are undergoing haemodialysis. Muscle growth may be impaired as a result of several catabolic conditions. Strength training was associated with important clinical outcomes including increased muscle strength and improved self-rated physical health and function.