Abstract
Background Synovial sarcoma is an aggressive soft tissue cancer of extremities mainly and rare in head and neck region, whereas rarest in ethmoidal sinus as only three cases have been reported till date. Case Reports We managed two cases of synovial sarcoma who presented with nasal obstruction, epistaxis, and swelling around the nasofacial region. Endoscopic nasal biopsy and immunohistochemistry markers confirmed synovial sarcoma in both the cases. While one case was managed by surgery and chemoradiation, the second patient received two cycles of ifosfamide-based chemotherapy and succumbed after 6 weeks of diagnosis. Conclusion Head and neck sarcomas are aggressive and carry a poor prognosis. Surgical resection with postoperative radiotherapy is the standard treatment. However, they have a high risk of recurrence and hence aggressive management and close follow-up is warranted for the optimal outcome.