Wilms’ tumor is the most common pediatric kidney cancer worldwide. Ninety percent of unilateral Wilms’ tumor cases occur in otherwise healthy patients and are regarded as sporadic, but 10% are associated with congenital anomalies or a tumor predisposition syndrome. Five to seven percent of Wilms’ tumor cases are found to be bilateral, involving both kidneys. Wilms’ tumor most often presents as an asymptomatic abdominal mass in children between 2 and 4 years old. Contrasted dual-phase CT scan of the abdomen and pelvis provides excellent anatomic evaluation of pediatric renal masses. Wilms tumor is typically chemosensitive, and either up-front radical nephroureterectomy or neoadjuvant chemotherapy is employed at diagnosis depending on local resources and protocol-driven management directives. The standard of care operation for unilateral Wilms’ tumor is radical nephroureterectomy with lymph node sampling, while bilateral nephron-sparing surgery is advocated for bilateral Wilms’ tumor. Difficult surgical scenarios including venous thrombectomy, nephron-sparing surgery, bilateral Wilms’ tumor, or resection of Wilms tumor in the setting of nephroblastomatosis benefit from experienced surgical teams. Increasing understanding of the molecular genetic etiology of non-Wilms’ tumor pediatric renal malignancies including renal cell carcinoma, rhabdoid tumor, clear cell sarcoma, congenital mesoblastic nephroma, multilocular cystic nephroma, and renal medullary carcinoma may lay the groundwork for new therapies in the future.