BACKGROUND: Pigmented villonodular synovitis (PVNS) is an infrequent benign tumor-like proliferative lesion developing from the synovial membranes of the joint, tendon sheath, and bursa. CLINICAL PRESENTATION: A 44-year-old woman with numbness on the right side of her face, severe headaches, and swelling in temporomandibular region is presented. On head and neck magnetic resonance imaging, an encapsulated mass approximately 2 cm was detected. The fine-needle aspiration biopsy resulted as suspicion of mesenchymal tumor. A complete resection with the capsule was performed over the temporal branch by monitoring of the facial nerve. The final histopathologic examination resulted as a giant cell tendon sheath tumor. CONCLUSION: Headache is not the main symptom in PVNS, but in severe pain spreading from the temporomandibular region, physical examination should be done carefully for slight swelling, and the possibility of pigmented villonodular synovitis should be considered. Because of the high recurrence rate, en bloc resection is necessary.
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