Given the shortcomings of previous literature reviews evaluating the effect and safety of exercise therapy in SSc, we aimed to carry out a systematic review of the literature specifically on this topic. A structured search strategy was performed in Medline (via PubMed) and other electronic databases from 1990 to 3 September 2019. Randomized controlled trials, observational designs, conference abstracts and trial registrations were included if they concerned SSc patients ≥18 years of age, exercise therapy and reported outcomes related to physical functioning. Nine articles were included. Four randomized controlled trials compared (a) hand exercises, (b) orofacial exercises, (c) aerobic exercises or (d) aerobic exercises plus resistance training with no exercise, demonstrating effects on hand function (a), maximum mouth opening (b), peak oxygen uptake (c + d) and quality of life. All five observational studies concerning hand, orofacial, aerobic and/or strengthening exercises reported improvements of hand function, mouth opening, aerobic capacity and/or muscle strength. In conclusion, the evidence on the effect and safety of exercise therapy in SSc is scanty.