Background: People with chronic kidney disease (CKD) experience a wide range of symptoms due to reduced kidney function. As the disease progresses these symptoms become more burdensome and often negatively affect a person's health-related quality of life (HRQoL). Objective: To examine the evidence of symptoms and HRQoL in CKD stages 1–5 and the relationships between these. Methods: Studies published in English from January 2008 to July 2018 using six databases (PubMed, MEDLINE, CINAHL, PsycINFO, Cochrane Library and JBI Library) were searched. Results: Thirteen studies were included in this review although only three had interventional designs. By considering symptom experience and HRQoL together, four studies found that HRQoL decreased when symptoms increased. Feeling washed out, fatigue and drowsiness were found to be the most common symptoms reported. Only two studies reported follow-up measurements although six studied how symptoms change over time. None of the studies examined the change of symptoms over time across the different CKD stages. The majority of studies showed a decline in physical HRQoL and improvement in mental HRQoL over time. Nutritional and exercise interventions showed some improvements in symptom experience and HRQoL. Conclusions: Only four studies were found that reported the relationship between symptoms and HRQoL of those with CKD. Of these, in view of symptom experience and HRQoL together, HRQoL decreased when symptoms increased. However, more research is warranted to establish a clear understanding of the relationship between symptoms and HRQoL in CKD to enable the design of appropriate interventions.