Nephroblastoma is the most common abdominal malignancy in pediatric patients. The tumors mainly occur within the first 5 years of life, and are especially common between the ages of 2 and 4 years. The number of left and right side tumors is similar, and 3-10% are bilateral, or occur simultaneously or successively. There is no difference between male and female gender, but most of the reports are slightly more in males than females. The tumor is made up of extremely nephroblast-like components because of the embryogenesis of the posterior kidney. The clinical manifestations and symptoms of nephroblastoma are closely related to the management of the disease, treatment planning and prognosis, which is very important. After years of observation and research, it is now recognized that congenital mesangioblastoma often occurs in the mammary stage, is well differentiated, and often has a benign course. Cystic nephroblastoma also has a benign course and a good prognosis. According to the International Society of Pediatric Oncology, which emphasizes the importance of cellular tissue type for prognosis, nephroblastoma can be divided into two types according to pathologic histology. How is nephroblastoma treated? Is the contralateral kidney normal before treatment? Does the tumor have distant metastasis? Can the tumor be removed? By reading CT, MRI before surgery, it is usually not difficult to answer this question. If necessary, digital subtraction angiography (DSA) of the renal artery is performed to understand the distribution of the mass and renal vessels to help assess the possibility of surgical resection. Radical nephrectomy is the surgical procedure commonly used in the treatment of nephroblastoma. In addition to removal of the primary tumor and kidney, retroperitoneal lymph node dissection is required. Partial nephrectomy or tumor enucleation is feasible when conditions allow and in the case of bilateral nephroblastoma. In addition to surgical resection, chemotherapy and radiotherapy are other important tools currently considered to improve their survival. In the stage without chemotherapy and radiotherapy, the overall survival rate of nephroblastoma is only 20-40%, while with the development of comprehensive treatment, the overall survival rate can reach 85-90%. Even more, the overall survival rate of children with FH pathology (good prognosis) can reach 94-100%, while the overall survival rate of children with UH pathology (poor prognosis) can also reach about 70%. The prognosis is mainly related to the stage of the disease and the type of pathology.