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Primary primitive neuroectodermal tumor of the kidney

Renal primitive neuroectodermal tumor (PNET) is a rare entity. It should be differentiated from other primary renal malignancies. The differentiation of the malignant small round cell tumors may be challenging; however, it is essential for better management of the patient. We report a 45-year-old male having complaints of pain in the abdomen, hematuria, and mass in the abdomen for 6 months. Abdominal and pelvic sonography finding showed cystic-solid, right renal mass suggestive of malignancy. On histopathology diagnosed as malignant small round blue cell tumor suggestive of primitive neuroectodermal tumor. On immunohistochemistry, CD99 positivity confirmed the diagnosis of primary PNET of the kidney. We are presenting this case for its rarity, clinical presentation, and pathological findings.