Abstract
Ewing's sarcoma is a small round cell tumor commonly involving long bones. Head and neck involvement is seen less frequently with maxillary sinus being the rarest site. Here we report a case of a12-year-old female patient presented with left side facial swelling for 3 weeks duration which was diffuse and hard on palpation. CT and MRI of Nose & para nasal sinus revealed a mass lesion in the left maxillary sinus infiltrating and eroding the orbital wall and extending intracranially. Biopsy from the lesion revealed Ewing's sarcoma with strong positivity for CD-99 marker. Patient was referred to radiotherapy due to extensive disease at younger age. The CT and MRI along with HPE and IHC markers enables early diagnosis and prompt treatment that leads to better survival and prognosis, as unlike Ewing's sarcoma of long bones, that metastasize early. The combined chemoradiotherapy is now the standard line of treatment along with close follow up for at least 2 years.