Chaudhary, RPChaudhary, M2026-04-232026-04-232016https://hdl.handle.net/20.500.14572/5896RP Chaudhary MS Pediatric Surgeon, Assoc.Professor, National Academy of Medical Sciences, Kanti's Children's Hospital, M Chaudhary MD Ophthalmologist, Assoc. Professor, IOM, Tribhuvan University.Abstract WAGR syndrome which includes Wilms' tumor, aniridia, genitourinary anomalies and mental retardation is a rare, sporadic, genetic disorder characterized by de nova deletion in the distal band of 11p13chromosome. Here, we report first case of WAGR from Nepal of a 5 year old male child with hypospadias, right Wilms' tumor and bilateral aniridia treated successfully by surgery and chemotherapy. Keywords: WAGR syndrome, Wilms' tumor, Hypospadias, Aniridia, Chemotherapyen-USWAGR syndromeWilms' tumorHypospadiasAniridiaChemotherapyArticle