Synovial chondromatosis (SC) in the temporomandibular joint (TMJ) is an uncommon entity, mostly when the involvement is bilateral. The authors report a rare case of bilateral SC, with a follow-up of 13 months, and a literature review. A 60-year-old Caucasian woman, with the chief complaint of pain for 6 years in the bilateral pre-auricular region, had a progressive clacking and discomfort on the left side during mouth opening. The panoramic image was suggestive of SC. The bilateral lesion was surgically removed by direct access. Histopathological examination confirmed the clinical diagnosis of bilateral SC. This article shows the importance of a multidisciplinary approach for the early diagnosis and appropriate treatment. Also, it encourages the referral of such cases to professionals with a greater familiarity with this entity.