Incidence rate of primary synovial chondromatosis of the shoulder joint makes 15–28 %. It is a rare benign lesion of synovial membrane of the joint featured by multiple foci of cartilaginous metaplasia in tissues of the membrane. Dif- ferential diagnostics should be conducted with the diseases, accompanied by formation of intra-articular loose bodies (secondary chondromatosis) at osteochondritis dissecans, osteochondral fracture of the humeral head, and tuberculous rheumatism. But the critical role belongs to histopathology report and molecular genetic analysis. Synovial chondroma- tosis most frequently afflicts one of the major joints (knee, hip, elbow, or shoulder). The pathology develops mainly in young men. Published descriptions of the development of this shoulder joint lesion are very sparse. Diagnostics becomes possible only on the grounds of histological examination of cartilage flaps removed from the joint. Present treatment of synovial chondromatosis includes removal of intra-articular cartilage bodies, either with open surgery or by arthro- scopic approach. The authors of the article present the clinical example of diagnostics and surgical treatment of the patient with primary synovial chondromatosis. The disease, aged more than 4 years, was accompanied with limited movements in the shoulder joint. Postoperative check-up examination established restored volume of movements and no pain syndrome in the joint. The patient came back to everyday routine and amateur sport. Therefore, adequate volume of surgical intervention excludes the recurrence of the disease in early and remote postoperative period. The patient is able to work and participate in routine physical activity.