Background: People with end-stage kidney disease (ESKD) suffer from multiple symptoms, which have a negative impact on their health-related quality of life (HRQoL). Educational and behavioural interventions are being developed for this population; however, the effect of these interventions is unclear. Aim: To evaluate the effectiveness of educational or behavioural interventions compared with standard care or alternative strategies on reducing symptoms and improving HRQoL in adults with ESKD receiving haemodialysis (HD). Methods: An effectiveness systematic review using Joanna Briggs Institute (JBI) procedures was conducted on experimental studies [randomised controlled trials (RCTs), pseudo-RCTs and quasi-experimental designs] published in English between January 2009 and July 2019. Studies were retrieved from CINAHL, PubMed, MEDLINE, Embase, PsycINFO, Cochrane Library (Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trial) and JBI databases. Effect size at 95% confidence interval was calculated where possible. Results: Eighteen studies involving 791 participants were included in this review. All studies involved behavioural interventions with the majority of studies (n = 11) targeting psychological symptoms. Interventions were categorised as either active or passive. Active interventions seemed to improve some physical symptoms, although there was very little evidence of improvements to HRQoL. Passive interventions tended to have a large effect on psychological symptoms and the mental health components of HRQoL. Conclusion: Due to great heterogeneity between studies, meta-analyses could not be conducted further limiting the evidence to inform practice. In addition, further research on educational interventions to teach self-management strategies for symptom management and to improve HRQoL in people with ESKD receiving HD are needed.