Background: Eating disorders are health problems that are particularly prevalent in adolescents and young adults. They are associated with considerable physical health and psychosocial morbidity, and increased risk of mortality. We set out to conduct a systematic review to determine their effect on physical fitness in the general population and on sport performance in athletes. Methods/Design: A systematic review of the relevant peer-reviewed literature was performed. For inclusion, articles retrieved from PubMed had to be published in English between 1977 and 2013. Wherever possible, methods and reporting adhere to the guidelines outlined in the PRISMA statement. Some additional studies were retrieved from among those cited in the reference lists of included studies and from non-electronic databases. Literature searches, study selection, method and quality appraisal were performed independently by two authors, and data was synthesized using a narrative approach. Results: Of the 1183 articles retrieved, twenty-nine studies met the inclusion criteria and were consequently analysed. The available data indicate that eating disorders have a negative effect on physical fitness and sport performance by causing low energy availability, excessive loss of fat and lean mass, dehydration, and electrolyte disturbance. Discussion: Although the paucity of the available data mean that findings to date should be interpreted with caution, the information collated in this review has several practical implications. First, eating disorders have a negative effect on both physical fitness and sport performance. Second athletics coaches should be targeted for education about the risk factors of eating disorders, as deterioration in sport performance in athletes, particularly if they are underweight or show other signs of an eating disorder, may indicate the need for medical intervention. However, future studies are needed, especially to assess the direct effect of eating disorders on sport performance.