UADT synovial sarcomas is an extremely rare clinical entity (only around 50 cases reported in the literature, with four more herein presented from our own surgical series), with no clear guidelines concerning its treatment. Resection should be aimed to an en-bloc removal of the tumor within uninvolved surgical margins. In fit patients, a conservative surgical approach should be preferred if it does not interfere with a complete resection and reasonable functional outcomes. Adjuvant treatments (radiotherapy and/or chemotherapy) are frequently needed in view of the aggressive behavior of such tumor, but they should be balanced according to patient's characteristics and tumor risk factors (grade, size, and previous treatments).