Abstract
Ewing's sarcoma is a part of a spectrum of the Ewing family of soft tissue tumors that have a primitive neuro-ectodermal origin most commonly originating from the appendicular and axial skeleton. Occurrence in the orbit is a rare presentation. case report a 15-year-old girl presented with painless unilateral swelling of the right eye since 4 months on histopathological examination came out to be a small round cell tumor with primitive neuroendocrine origin. she was started on chemotherapy with VAC+IE regimen as induction chemotherapy but as the disease was progressive and not responding after 17 cycles of alternating VAC+IE second-line chemotherapy with irinotecan and temozolomide was started and after three cycles surgery was deferred i/v/o intracranial extension so she was treated with external beam radiotherapy 59.4gy in 33#'s at 1.8gy/# with concurrent irinotecan and temozolomide with complete response after radiotherapy. she was on follow-up for 4 months and was later diagnosed with secondary deposits in the lung which she could not cave in and expired.
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