Abstract: Bilateral Wilms' tumor (BWT) affects approximately 5% of children with WT. The goal of treatment in BWT is to preserve normal renal function as much as possible, in order to postpone and reduce the risk of end-stage renal disease and other long-term complications. For neoadjuvant chemotherapy, vincristine/dactinomycin/doxorubicin is used in cases with favorable histology. Cases with diffuse anaplasia or other unfavorable histology receive an intensified regimen, including additional agents such as ifosfamide, cyclophosphamide, carboplatin, and others. Nephron-sparing surgery is performed to preserve long-term renal function. Radiotherapy is given according to local staging, when margins or lymph nodes are positive, or tumor spillage has occurred, or pathological type is anaplasia. The survival rate of relapsed BWT is low. Some experts suggest that patients with recurrent BWT are most likely to benefit from high-dose therapy including stem-cell transplantation, but this remains controversial.