Peripheral primitive neuroectodermal tumour/Ewing’s sarcoma (PNET/EWS) is an aggressive type of sarcoma. It is second most common sarcoma in the paediatric–young adult age range. However it is extra ordinarily rare primary tumour in the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Histopathology and immunohistochemistry are useful in confirmation of the condition. Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and early metastasis. Lung, liver, bone being common metastatic sites. However we present case of primary Ewing’s Sarcoma with Orbital metastasis which has not been much reported.

Keywords: Extra Osseous Ewing’s Sarcoma; Orbital metastasis