Systemic sclerosis (SSc) is a multi-organ, systemic connective tissue disease, which affects the lungs, heart, gastrointestinal tract, kidneys, skin, and musculoskeletal system. Musculoskeletal involvement is observed in 40–90% of patients with SSc. During the disease, any structure of the musculoskeletal system, such as bones, joints, tendon sheaths, tendons, and muscles, may be affected. The most common symptoms include joint pain, arthritis, tendinitis leading to tendon rupture, acro-osteolysis, calcinosis, myalgia, and myositis. Osteo-articular complications and changes in the soft tissues of the hand lead to finger contracture, which causes deterioration of the patients’ quality of life and disability. To sum up, a more detailed understanding of the aetiology leading to progressive changes in the musculoskeletal system may contribute to the introduction of new therapeutic options, and thus improve the quality of life and reduce disability in patients with SSc.