Musculoskeletal tumors are not usually encountered at the skull base. Benign lesions such as cholesterol granuloma, cholesteatoma, fibrous dysplasia, and Paget disease are common examples of such tumors arising from the bone of the cranial base. The authors report a case involving an adult male patient with the rare finding of pigmented villonodular synovitis of the temporomandibular joint. This case is reported as an example of another lesion to consider in the differential diagnosis. Due to the complexity of the anatomy at the cranial base, radical resection as recommended for the usual sites of occurrence in major joints is problematic. Because of the reported high recurrence rates at other sites, as radical excision as possible with vigilance for recurrence is recommended.