Synovial sarcoma (SS) is an extremely rare head and neck malignancy in the pediatric population.

Preoperative diagnosis of SS is challenging because of its nonspecific symptomatology, low clinical morbidity at presentation, lack of specific radiologic features, and physicians unfamiliarity.

We present this case to emphasize the correlation between histopathology diagnosis and clinical judgment to perform optimal treatment (surgical, chemo and/or radiotherapy) with preservation of pharyngeo-laryngeal function in the pediatric population.