Children born with very rare complex of congenital developmental abnormalities such as aniridia, genito-urinary malformations and mental retardation have higher than 30 percent chance of having a concomitant Wilms' tumor (WAGR Syndrome). This is listed as a rare disease by the Office of Rare Diseases of the National Institutes of Health (NIH).

We report the case of a 20 month old baby diagnosed with WAGR Syndrome who underwent exploratory laparotomy, ipsilateral radical nephrectomy and contralateral partial nephrectomy and contralateral partial nephrectomy. A horseshoe kidney was incidentally diagnosed intra- operatively which is not an uncommon finding as reported by the National Wilms' Tumor Study Group (NWTSG).

This paper aimed to document and reported this rare condition which was completely worked up and address surgically by the subspecialties of pediatric surgery together with the pediatric urology.