The popularity of running is still growing and, as participation increases, the incidence of running-related injuries will also rise. Iliotibial band syndrome (ITBS) is the most common injury of the lateral side of the knee in runners, with an incidence estimated to be between 5% and 14%. In order to facilitate the evidence-based management of ITBS in runners, more needs to be learned about the aetiology, diagnosis and treatment of this injury. This article provides a systematic review of the literature on the aetiology, diagnosis and treatment of ITBS in runners. The Cochrane Library, MEDLINE, EMBASE, CINAHL, Web of Science, and reference lists were searched for relevant articles. Systematic reviews, clinical trials or observational studies involving adult runners (>18 years) that focused on the aetiology, diagnosis and/or treatment of ITBS were included and articles not written in English, French, German or Dutch were excluded. Two reviewers independently screened search results, assessed methodological quality and extracted data. The sum of all positive ratings divided by the maximum score was the percentage quality score (QS). Only studies with a QS higher than 60% were included in the analysis. The following data were extracted: study design; number and characteristics of participants; diagnostic criteria for ITBS; exposure/treatment characteristics; analyses/outcome variables of the study; and setting and theoretical perspective on ITBS. The studies of the aetiology of ITBS in runners provide limited or conflicting evidence and it is not clear whether hip abductor weakness has a major role in ITBS. The kinetics and kinematics of the hip, knee and/or ankle/foot appear to be considerably different in runners with ITBS to those without. The biomechanical studies involved small samples, and data seem to have been influenced by sex, height and weight of participants. Although most studies monitored the management of ITBS using clinical tests, these tests have not been validated for this patient group. While the articles were inconsistent regarding the treatment of ITBS, hip/knee coordination and running style appear to be key factors in the treatment of ITBS. Runners might also benefit from mobilization, exercises to strengthen the hip, and advice about running shoes and running surface. The methodological quality of research into the management of ITBS in runners is poor and the results are highly conflicting. Therefore, the study designs should be improved to prevent selection bias and to increase the generalizability of findings.