Ewing's sarcoma/primitive neuroectodermal tumor is a rare entity belonging to a family of neoplasms of neuroectodermal origin. These highly aggressive neoplasms overwhelmingly affect children and young adults. A quarter of Ewing's sarcomas occur principally in the soft tissues with primary involvement of the perinephric fat and kidney being exceptionally rare. We present a 38-year-old man with primary Ewing's sarcoma of the perinephric fat and kidney diagnosed 2 years earlier who subsequently developed an infiltrating left renal and perinephric mass.
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