Abstract
Extraosseous Ewing's sarcoma (EES) of the larynx is an exceptionally rare malignancy, with very few cases reported in the literature. Its clinical behavior and optimal management remain poorly defined. We report the case of a 14-year-old patient who presented with hoarseness of voice. Imaging and histopathological evaluation confirmed a diagnosis of laryngeal EES. The patient was treated with systemic chemotherapy followed by radiation therapy, resulting in a favorable clinical and radiological response. EES can occur across a broad age range and may demonstrate aggressive local growth and early metastatic potential. Due to the rarity of laryngeal involvement, standardized treatment guidelines are lacking, and management is often extrapolated from Ewing's sarcoma at other sites. This case highlights the aggressive nature of laryngeal EES and underscores the importance of early diagnosis, comprehensive metastatic workup, and a multidisciplinary treatment approach. Additional case reports are needed to define the prognosis better and optimize management strategies.