Wilms Tumor (WT) is an extremely rare neoplasia in adults. It represents 95% and 1% of all renal tumors, in adults and children respectively. Clinical symptoms are also distinct between adults and children. In adults, symptoms are usually abdominal pain and hematuria, on the other hand, pediatric patients frequently present with an asymptomatic abdominal mass. The recommended treatment for WT in adults follows protocols established for pediatric patients. Currently, the 5-year overall survival rate for adults with WT is of approximately 90%. The adoption of multimodal therapy systematized, and proposed by current protocols, allowed the results to become much more robust, with a significant improvement from less than 30% to 90%. However, recent reviews suggest a worse outcome for adults when compared to children, even when submitted to the same treatment regimen, and comparable according to histology and tumor stage.We present a case of a 26 years-old male patient, who underwent surgery to treat a solid renal mass. Pathological report demonstrated a Wilms tumor. The patiente also received adjuvant chemotherapy with dactinomycin and vincristine. After 12 months follow-up, patient has no evidence of disease.