Wilms tumor is very rare in adults. Even more infrequent is an adult Wilms tumor with an extensioninto the central nervous system.Reported here is a case of an adult Wilms tumor in a 38-year-old female. She was referred to theJRRMMC with a 2 month history of intermittent hematuria associated with a rapidly enlargingabdominal mass and right-sided facial asymmetry. Abdominal computed tomography revealed a largemass inthe rightkidney. CranialMRI showedmultiple brainmetastases. Thepatient underwentrightradicalnephrectomy.Pathologicalanalysisdemonstratednephroblastoma.Thepatientwasdischargedunremarkable and underwent adjuvant chemotherapy. After 2 months, the patient succumbed to thedisease.Adult Wilms tumor presents almost similarly with renal cell carcinoma and there is no definitivediagnostictesttoconfirmitpre-operatively.Eventhoughit’sararetumor,itshouldalwaysbeincludedin the differential diagnosis for any renal tumor.