Synovial chondromatosis is a cartilaginous metaplasia of the mesenchymal remnants of the synovial tissue of the joints. Its main characteristic is the formation of cartilaginous nodules in the synovium and inside the articular space (loose bodies). Synovial chondromatosis mainly affects big synovial joints such as the elbow and knee and is uncommon in the temporomandibular joint. The main symptoms are pain, limitation of jaw movement, crepitation, and inflammation. Diagnosis is made by panoramic radiograph, computed tomography scan, and mainly magnetic resonance imaging. Surgery is the therapeutic choice. The authors describe their experience in the treatment and in the follow up of a patient with unilateral synovial chondromatosis.