Nephroblastoma is the most common malignant renal tumor in pediatric patients. It was first reported by Wilms, so it is also known as “Wilms’ tumor”. In the past 20 years, due to the development of comprehensive treatment measures such as surgery, chemotherapy and radiotherapy, as well as the National Wilms’ Tumor Study Group (NWTSG) and the International Society of Pediatric Oncology (SIOP), the prognosis for Wilms’ tumor is relatively good. The application of multicenter research results such as the National Wilms’ Tumor Study Group (NWTSG) and the International Society of Pediatric Oncology (SIOP) in Europe has significantly improved the efficacy, gradually reduced the complications in low-risk patients, and further improved the long-term survival rate in high-risk patients. The incidence of nephroblastoma in infants and children is about 1-2 per million population, and it occurs in infants and children aged 1-3 years. The tumor originates from the posterior renal blastoderm and is an embryonal tumor arising from the residual immature kidney. It may be associated with urogenital malformations, limb deformities or mental retardation. It may be associated with genes such as WT1, WT2, and P53, or with genetic factors such as Denys-Drash, Beckwith-Wiedemann, and WAGR syndromes. An abdominal mass is the most common presentation and can be found incidentally during a change of clothes or bathing. 95% of patients have a mass palpated at the first visit. The mass is located in the upper abdomen on the side of the ribs, with smooth surface, moderate firmness, no pressure pain, and may have some mobility in the early stage. When the tumor is rapidly enlarging/huge, it may produce compression symptoms, shortness of breath, lack of appetite, emaciation and irritability. About 1/3 of children have abdominal pain, ranging from local discomfort and mild pain to severe pain and colic, and if accompanied by fever, anemia and hypertension, it often indicates subperitoneal hemorrhage. There is also acute abdomen caused by tumor rupture. About 25% of children have microscopic hematuria and 10-15% have carnal hematuria. Hematuria is most often induced by minor trauma to the enlarged kidney or related to tumor invasion of the renal pelvis and does not indicate advanced tumor. Increased blood pressure occurs in about 30% of cases, either due to renin production by tumor cells or high renin-angiotensin due to renal vascular embolism or renal artery compression. Often, blood pressure returns to normal after tumor removal. It may be combined with acute renal failure, varicocele, and hypoglycemia. Erythrocytosis is rare and the cause may be related to tumor production of erythropoietin. Combined with nephrotic syndrome, it is called Wilms’ nephritis. Inferior vena cava obstruction may lead to hepatomegaly and ascites. If it invades the right atrium, it may lead to congestive heart failure. Hematogenous metastasis may spread to all parts of the body, with pulmonary metastasis being the most common, which may cause cough, pleural effusion, chest pain, hypothermia, anemia and cachexia. Before treatment, we should know whether the opposite kidney is normal? Does the tumor have distant metastasis? Can the tumor be removed? It is generally not difficult to answer this question by reading CT and MRI before operation. If necessary, digital subtraction angiography (DSA) of the renal artery will be 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 more than 92%, while the overall survival rate of children with UH pathology (poor prognosis) can also reach more than 60%. The prognosis is mainly related to the stage of the disease and the type of pathology.