[Purpose] The aim of the study was to conduct a systematic review about rehabilitation treatment of hypermobile Ehlers-Danlos syndrome, according to Evidence Based Medicine. [Methods] A systematic search has been conducted in following database: PubMed, PEDro, Cochrane, EDS Base Index, TRIP and CINHAL; no time restrictions were adopted. PICO method was employed to formulate the clinical query. Hypermobile Ehlers-Danlos syndrome and physiotherapy were the main keywords of the research. [Results] 1,869 articles emerged from the primary search. After duplicates removal, 1,709 papers have been screened by title and abstracts and then 1,698 were excluded following the inclusion criteria. 11 papers have been admitted to the last stage of the review and have been evaluated in their full-text version. Only one cohort study met the review’s final step selection criteria. One cohort study on the efficacy of an integrated physiotherapeutic and cognitive behavioural treatment was evaluated by means of Newcastle-Ottawa Quality Assessment score and resulted of poor evidence. [Conclusion] At the present time, there is no Evidence Based Medicine literature on hypermobile Helers-Danlos syndrome rehabilitation. Clinical studies with high level of evidence are necessary in order to assess the efficacy of physiotherapeutic approaches.