Synovial chondromatosis (SC) in temporomandibular joint is a rare benign disorder characterized by cartilaginous metaplasia of the mesenchymal remnants of the synovial tissue. The etiology of the disease is unclear but may be associated with trauma, overuse, local infection, and embryologic disturbance. SC does not spontaneously resolve and respond to nonsurgical treatment. SC should be differentially diagnosed with other temporomandibular joint disorders such as arthralgia or osteoarthritis because surgery should be done for managing it. However, primary diagnosis of SC is not easy because of nonspecific symptoms and signs. For the patients with unsuccessful conservative treatment response, especially accompanied by crepitus, preauricular swelling or posterior open bite, computed tomography/cone-beam computed tomography or magnetic resonance imaging should to be performed to exclude SC. We discussed the importance of the early diagnosis and surgical treatment of SC from this case.