IntroductionLow health literacy is widely reported in people with chronic kidney disease (CKD) and has been associated with reduced disease self-management, poor health outcomes, increased mortality and poorer quality of life. However, these associations are still not well understood.MethodsElectronic-based systematic searches were performed to identify studies examining associations between health literacy and self-management behaviours and/or health outcomes in patients with CKD. A tabular and narrative synthesis of the data was performed. Meta-analysis was not appropriate due to the heterogeneity of study designs and methods.ResultsSearches identified 48 studies that met the inclusion criteria. A total of 41 published articles, six conference abstracts, and one thesis were included. Of the 48 studies, 11 were cohort and 37 were cross-sectional. In total there were 25,671 patients; 16,952 from cohort studies. Median study sample size was 159 (IQR 92–275). Study quality was high (5), moderate (24) and poor (19). Thirteen measures of health literacy were used. Despite the limitations of the available evidence, there appear to be consistent relationships between higher health literacy and favourable self-management behaviours for patients with CKD. Definitive relationships between health literacy and patient outcomes are far less clear and remain incompletely understood.DiscussionConclusive evidence describing a causal link between health literacy and patient outcomes remains limited, but for many outcomes, a consistent association is described. In addition to associations with mortality, hospitalisation and clinical events, there were consistent associations between health literacy and favourable self-management behaviours which could support the development of patient education aimed at improving health literacy.Graphical abstractSupplementary InformationThe online version contains supplementary material available at 10.1007/s40620-022-01537-0.