© 2019, Japanese Society for Oral and Maxillofacial Radiology and Springer Nature Singapore Pte Ltd. A case of tophaceous pseudogout (i.e., calcium pyrophosphate dihydrate crystal deposition disease) in the temporomandibular joint (TMJ) extending into the cranium is reported. A 59-year-old woman was referred to hospital with swelling and pain in the left cheek, and with trismus. Computed tomography imaging revealed a large, granular, calcified mass surrounding the left condylar head, partly destroying the cortex of the condylar head, and extending into the cranium by destroying the glenoid fossa. Magnetic resonance imaging revealed that the soft-tissue mass was of low-signal intensity on T1- and T2-weighted images, and was enhanced after intravenous injection of gadolinium. The mass was clinically and radiographically suspected to be a neoplastic lesion or a synovial osteochodromatosis. However, histological analysis demonstrated that the mass contained granulomatous lesion due to multiple nodular deposits of numerous rod-shaped and rhomboid crystals, which verified the diagnosis of tophaceous pseudogout. The lesion was excised surgically using a preauricular approach. Neither radiographic nor clinical examination demonstrated any signs of mass recurrence in the long-term 8- and 14-year postoperative recall examinations. Tophaceous pseudogout is a rare benign arthropathy that presents with clinical and radiographic features mimicking neoplastic conditions of the TMJ. Therefore, it is recommended that tophaceous pseudogout is considered in the differential diagnosis when a calcified mass lesion of the TMJ is encountered.