Synovial chondromatosis is an uncommon benign disorder of the synovial membrane ofjoints, tendon sheaths, or bursae characterized by the formation of multiple cartilaginous nodules or osseus loose bodies. It is usually a monoarticular disease, and 33 different localizations have been described until now. The aim of this review article is to present the newest knowledge on the etiology, diagnosis, differential diagnosis, and management of synovial chondromatosis. Malignant transformation to chondrosarcoma is an unusual but possible complication. It is closely connected with recurrence rate and usually occurs many years after surgical treatment. More specific details related to surgical treatment of most often affected joints, i.e. shoulder, elbow, hip, knee, and ankle are reported as well at the end of this review article.