Tumor thrombosis of the inferior vena cava in children with nephroblastoma is a relatively rare complication that requires a multimodal approach to treatment and involves many specialists. This condition occurs in 10% of children with Wilms tumor but cases when a tumor thrombus extends to the orifices of the hepatic veins and more cranially, are much less common. In most patients, neoadjuvant chemotherapy can significantly reduce the size of a tumor thrombus, which may eliminate the need for revision surgery of the inferior vena cava. Due to the rarity of this clinical condition, the optimal surgical strategy for tumor thrombosis of the inferior vena cava in children has not been fully defined yet. Here, we present a clinical case of a child with locally advanced stage 4 Wilms tumor of the right kidney and tumor thrombosis of the inferior vena cava that extended mostly in the caudal direction, to the confluence of the iliac veins. The patient received 6 weeks of neoadjuvant therapy with AVD (actinomycin D, vincristine, doxorubicin) and underwent nephrectomy for local control, with prosthetic replacement of the terminal sections of the common iliac veins as well as of the inferior vena cava up to its subhepatic segment, and implantation of the left renal vein. In the early postoperative period, the child developed thrombosis of the prosthesis, without hemodynamically significant abnormalities. At the time of writing, the patient had been followed up for 12 months and was considered to be in remission. The patient’s parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.