UNLABELLED: Wilms tumor is rare in adults. Though the approach to diagnosis and treatment of adult Wilms tumor (AWT) is closely modeled on recommendations for childhood Wilms tumor, views differ on how aggressive the treatment should be. We report a case of a 37-year-old with Stage III favorable histology AWT. A radical nephrectomy was performed and the patient was due for chemotherapy. Recent advances, controversies and current recommendations in the treatment of AWT are discussed. KEYWORDS: Adult; Wilms tumor; Kidney.