There is a lack of knowledge about the practice of physical activity, exercise, and sports in people with skeletal dysplasia (SD). This study aimed to characterize the physical fitness of people with SD; investigate the benefits of physical activity, exercise, or sports programs for people with SD; identify the adapted physical activities that can be prescribed to individuals with SD; and identify the most common and effective structural characteristics and guidelines for the evaluation of individuals with SD and corresponding activity prescriptions. Electronic searches were carried out in the PubMed, Scopus, SPORTDiscus, Psycinfo, and Web of Science databases in October 2021 and March 2022 and included papers published until 3 March 2022. The search strategy terms used were “dwarfism”, “dwarf”, “skeletal dysplasia”, “achondroplasia”, “pseudoachondroplasia”, “hypochondroplasia”, “campomelic dysplasia”, “hair cartilage hypoplasia”, “x-linked hypophosphatemia”, “metaphyseal chondrodysplasia schmid type”, “multiple epiphyseal dysplasia”, “three M syndrome”, “3-M syndrome”, “hypophosphatasia”, “fibrodysplasia ossificans progressive”, “type II collagen disorders”, “type II collagenopathies”, “type II collagenopathy”, “physical activity”, “exercise”, “sport”, “training”, and “physical fitness”, with the Boolean operators “AND” or “OR”. After reading the full texts of the studies, and according to previously defined eligibility criteria, fifteen studies met the inclusion criteria; however, there was not a single intervention study with physical exercise. Several cross-sectional, review, or qualitative studies presented a set of essential aspects that future intervention studies can consider when evaluating, prescribing, and implementing physical exercise programs, as they allowed the physical characterization of the SD population. This study demonstrated an apparent scarcity in the literature of experimental studies with physical exercise implementation in the SD population.