Ewing's sarcoma, a primitive neuroectodermal tumour is a rare entity belonging to a family of neoplasms of neuroectodermal origin. These highly aggressive neoplasms primarily affect older children and young adults. They primarily involve the bones or soft tissues. A quarter of Ewing's sarcomas occur principally in the soft tissues with primary involvement of the kidney being exceptionally rare. We hereby, present a case of 6 years old girl child with primary Ewing's sarcoma of the kidney who presented with abdominal mass and posed much diagnostic challenge. The patient underwent complete surgical excision of tumour, and is being treated with aggressive adjuvant chemotherapy.
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