Synovial sarcoma (SS) is a malignant soft-tissue sarcoma primarily affecting the lower extremities of young adults. Synovial sarcoma occurring in the head and neck region is rare.

We report a case of a 32-year-old female with a biphasic synovial sarcoma, showing extensive calcification, arising in the temporomandibular joint (TMJ). For effective diagnosis and treatment of synovial sarcomas in the TMJ, we reviewed relevant cases reported in the English literature and further discussed the clinicopathologic features, differential diagnosis, treatment strategies and prognosis.

The typical slow growth and clinical symptoms of SS in the TMJ region may often be misdiagnosed as temporomandibular disorders (TMD) or other benign neoplasms. Differential diagnosis to include primary parotid tumours should be considered and immunohistochemistry along with molecular studies can be helpful to verify the diagnosis. A multimodal treatment approach consisting of a wide surgical excision and postoperative radiotherapy should be taken into consideration.