Wilms' tumor is the most common childhood renal tumor. This article describes the epidemiology, histopathologic features, and clinical manifestations of Wilms' tumor along with the spectrum of imaging findings using different modalities. The distinguishing features of other renal tumors encountered in children, such as clear cell sarcoma, rhabdoid tumor, congenital mesoblastic nephroma, multilocular cystic renal tumor, renal cell carcinoma, and angiomyolipoma are also reviewed.