Primitive neuro-ectodermal tumors (PNETs) are seen mostly at central nervous system. Peripheral localizations are rare. PNETs are belong to Ewing sarcoma family. Ewing sarcoma has two subtypes as originated from bones or from another organ. Renal PNET is a very rare tumor. Peripherally localized PNETs originated from kidney, usually found at young men, and have poor prognosis with aggressive behavior. A 28-year-old man referred to our clinic with left flank pain and gross hematuria. Radiological investigations yielded gross renal mass compatible with malignancy. Radical nephrectomy was performed. Histopathological diagnosis was PNET/Ewing sarcoma with findings of large and hyperchromatous nuclei, narrow cytoplasms, spindle shaped cells- but mostly rosette formation- and strong straining with CD99. Chemotherapy was given to the patient. At the end of the chemotherapy, there no recurrence was detected by radiological investigations.
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