Synovial Chondromatosis (SC) of the Temporomandibular joint (TMJ) is a rare disorder with an indolent clinical course that leads to nonspecific symptoms, dysfunction, and anatomic distortion of the TMJ. A case is described of SC of the TMJ in a 56-year-old female that presented with pain in the right side of her face. The SC diagnosis was suggested after correlating the clinical and imaging findings. Open TMJ surgery was performed to remove the lesion, synovectomy, repair the perforated articular disc, and reposition the condyle into the glenoid fossa. At the 3-year follow-up appointment, the patient's condition was significantly better with stable occlusion, elimination of pain, and an incisal opening of 35 mm. There was no clinical or radiographic evidence of recurrence. This paper review the clinical, radiological, cytological, and pathological features of this entity and relates the differential diagnosis to other cartilaginous lesions and neoplasms of the TMJ. Accurate diagnosis requires the combination of clinical, radiological and histological studies. Awareness of this disorder is important to provide adequate care and avoid delayed treatment.