Wilms tumor (nephroblastoma) is rare in adults. We present a case of 28-year old female with Wilms tumor diagnosed with pre-operative abdominal magnetic resonance imaging, histopathological analysis and immunohistochemistry. She had relapse tumor two years after primary open radical nephrectomy. She was managed with chemotherapy with the ICE regimen (ifosfamide, carboplatin, and etoposide) for six cycles with partial response and excellent functional status