The authors report an unusual case of painful synovial chondromatosis originated in the inferior compartment of the temporomandibular joint (TMJ) with articular disc involvement in a 56-year-old woman with complaint of severe pain in the right preauricular region. Magnetic resonance images showed advanced destruction of the right articular disc anteriorly displaced, condylar erosion, and distinct nodules within an extremely expanded inferior joint compartment with large amount of fluid, as well as a large TMJ effusion. A scintigraphy showed elevated bone uptake in the right TMJ, demonstrating intense bone remodeling activity in the region. After a right internal maxillary artery embolization, the patient underwent open surgery. The intraoperative procedures, including articular disc removal, condylar remodeling, and replacement of the articular disc, are described in detail. Synovial chondromatosis of the TMJ is a rare disease, especially when it affects the inferior compartment and the articular disc. Initial diagnosis is challenging and imaging techniques (magnetic resonance imaging and scintigraphy) play an important role in identifying signs, making accurate diagnosis, and offering additional information not available with conventional imaging, such as TMJ inflammation or remodeling. In these patients, open surgery may be considered a definitive treatment, since the postoperative recurrence rate is very low.