BACKGROUND: A systematic appraisal of the worldwide incidence and prevalence rates of UEDs available in scientific literature was executed to gauge the range of these estimates in various countries and to determine whether the rates are increasing in time. METHODS: Studies that recruited at least 500 people, collected data by using questionnaires, interviews and/or physical examinations, and reported incidence or prevalence rates of the whole upper-extremity including neck, were included. RESULTS: No studies were found with regard to the incidence of UEDs and 13 studies that reported prevalence rates of UEDs were included. The point prevalence ranged from 1.6-53%; the 12-months prevalence ranged from 2.3-41%. One study reported on the lifetime prevalence (29%). We did not find evidence of a clear increasing or decreasing pattern over time. The case definitions for UEDs used in the studies, differed enormously. Therefore, it was not possible to pool the data. CONCLUSION: There are substantial differences in reported prevalence rates on UEDs. Main reason for this is the absence of a universally accepted way of labelling or defining UEDs. If we want to make progress in this field, the first requirement is to agree on unambiguous terminology and classification of EUDs.