The group of genetic degenerative ataxias shares the same feature of gradual deterioration in balance and coordination. However, no cure is yet available for this group of disorders, while rehabilitation remains a cornerstone in the current therapy. This review aims to present a summary of the current knowledge of balance and coordination training in patients with inherited degenerative ataxia and to discuss the training effectiveness accordingly.A comprehensive search was performed in 5 electronic databases (i.e., Cochrane Library, PEDro, EMbase, PubMed and MEDLINE) to identify the related publications from January, 1999 to January, 2020. Methodological quality was assessed using the Scottish Intercollegiate Guidelines Network (SIGN) grading system and the PEDro scale.A total of 33 out of 515 studies met the eligibility criteria, and were categorized and discussed by their training methods including: (1) conventional physical/occupational therapy, (2) virtual reality/videogame-based training, and (3) adapted physical activity. Despite the substantial variation among included studies, most patients achieved significant improvement in the aspect of balance and coordination following individually-tailored rehabilitation programs. The effects of training showed a relative consistency regardless of the functional dependency level on admission.Balance and coordination training, especially the conventional physical/occupational therapy, is able to improve the balance and coordinative function of patients with genetic degenerative ataxia, but more high-quality studies are needed to formulate recommendations for clinical practice.