The failure of arteriovenous fistulas (AVF) to mature is a major problem in patients with kidney failure who require haemodialysis (HD). Preoperative planning is an important factor in increasing functional AVF. Upper limb exercise has been recommended to gain AVF maturation. Studies of pre- and post-operative upper limb exercises in patients with kidney failure patients have been reported; however, the optimal program for this population is unknown due to inconsistent results among these programs. We aimed to determine if upper limb exercise would be beneficial for AVF maturation (prior to and post AVF creation) in patients with kidney failure and to improve AVF outcomes. This review also aimed to identify adverse events related to upper limb exercise. We searched the Cochrane Kidney and Transplant Register of Studies up to 15 March 2022 through searches of CENTRAL, MEDLINE, and EMBASE, conference proceedings, the International Clinical Trials Register (ICTRP) Search Portal and ClinicalTrials.gov, and other resources (e.g. reference list, contacting relevant individuals, and grey literature). We included randomised controlled trials (RCTs) and quasi-RCTs, comparing upper limb exercise training programs with no intervention or other control programs before or after AVF creation in patients with kidney failure. Outcome measures included time to mature, ultrasound and clinical maturation, venous diameter, blood flow in the inflow artery, dialysis efficacy indicator, vascular access function (functional AVF), vascular access complications, and adverse events. Study selection and data extraction were taken by four independent authors. Bias assessment and quality assessment were undertaken independently by two authors. The effect estimate was analysed using risk ratio (RR) with 95% confidence intervals (CI) for dichotomous data, or mean difference (MD) or standardised mean difference (SMD) for continuous data. Confidence in the evidence was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. Nine studies (579 participants) were included, and seven studies (519 participants) conducting post-operative exercise training could be meta-analysed. Three comparisons were undertaken: (i) isotonic exercise training versus no intervention; (ii) isometric versus isotonic exercise training; and (iii) isotonic (high volume) versus isotonic exercise training (low volume). Due to insufficient data, we could not analyse pre-operative exercise training. Overall, the risk of bias was low for selection and reporting bias, high for performance and attrition bias, and unclear for detection bias. Compared to no intervention, isotonic exercise training may make little or no difference to ultrasound maturation (2 studies, 263 participants: RR 1.09, 95% CI 0.94 to 1.25; I² = 0%; low certainty evidence), but may improve clinical maturation (2 studies, 263 participants: RR 1.14, 95% CI 1.02 to 1.27; I² = 0%; low certainty evidence). Compared to isotonic exercise training, isometric exercise training may improve both ultrasound maturation (3 studies, 160 participants: RR 1.56, 95% CI 1.21 to 2.00; I² = 22%; low certainty evidence) and clinical maturation (3 studies, 160 participants: RR 1.80, 95% CI 1.18 to 2.76; I² = 53%; low certainty evidence). Venous diameter (3 studies, 160 participants: MD 0.84 mm, 95% CI 0.45 to 1.23; I² = 0%; low certainty evidence) and blood flow in the inflow artery (3 studies, 160 participants: MD 140.62 mL/min, 95% CI 38.72 to 242.52; I² = 0%; low certainty evidence) may be greater with isometric exercise training. It is uncertain whether isometric exercise training reduces vascular access complications (2 studies, 110 participants: RR 2.54, 95% CI 0.38 to 17.08; I² = 47%; very low certainty evidence). It is uncertain whether high volume isotonic exercise training improves venous diameter (2 studies, 93 participants: MD 0.19 mm, 95% CI -0.75 to 1.13; I² = 34%; very low certainty evidence) or blood flow in the inflow artery (1 study, 15 participants: MD -287.70 mL/min, 95% CI -625.99 to 60.59; very low certainty evidence) compared to low volume isotonic exercise training. None of the included studies reported time to mature, dialysis efficacy indicator, vascular access function, or adverse events. Our findings suggest that the current research evidence examining upper limb exercise programs is of low quality, attributable to variability in the type of interventions used and the overall low number of studies and participants.