Wilms’ tumor is one of the most common childhood solid malignancies, which accounts for almost 95% of renal malignancies in pediatrics, and classically arises from primitive metanephric cells. Exceptionally it may occur at places other than kidneys. The estimated rate of nephroblastoma outside the kidneys is almost 0.5 to 1% of Wilms’ tumor cases. Extrarenal Wilms’ tumor occurs mostly in childhood. In this article, we report a 3-year-old girl who first presented with spinal dysraphism and a mass in the lumbar spinal cord with a histopathological diagnosis of nephrogenic rest, and after one year, a Wilms tumor arose in this location. When extrarenal wilms tumor located in the spine is unlikely to be suspected preoperatively because it is embryologically less intuitive. Thus, we report this case of a congenital Wilms tumor associated with spinal dysraphism to increase awareness and describe this malignant tumor’s clinical outcome.