Nephroblastoma is the most frequent renal tumor in childhood population. Rarely, it can occur in adults. In this case, the diagnosis is frequently challenging for pathologists. No standard guidelines are available for this neoplasm in adults. It needs multidisciplinary collaboration for optimal management. Herein we report a 26-year-old man presenting with a non-metastatic right nephroblastoma. He underwent a radical nephrectomy. He is currently in complete remission after a follow-up of 18 months.
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