Chronic kidney disease (CKD) is associated with a high risk of cognitive impairment (CI). Both vascular and metabolic factors are implicated in the causation of CI in CKD. The traditional risk factors for CI are more prevalent in CKD and interact reciprocally. CI in CKD is associated with reduced functional capacity, poor quality of life and mortality. Cognition declines significantly after initiation of haemodialysis (HD). Repeated cerebral insults related to intra-dialytic haemodynamic instability may be responsible for the rapid, step-wise decline in cognition observed in HD patients. Cognitive interventions used in the general population have not been adequately tested in CKD. Exercise interventions are likely to be beneficial based on biological plausibility and pilot trial data. Cooled HD may be beneficial in HD patients but needs substantive trial data to support it. Cognition testing should be routinely offered to CKD patients. There is a need for further research into the underlying causes of CI in CKD with a view to developing therapeutic interventions.