Nephroblastoma, also known as renal embryonal tumor or Wilms tumor, is the most common malignant tumor of the kidney in children, accounting for approximately 8% of all solid tumors in children. The age of onset is mainly between 2-4 years. Most Wilms’ tumors are considered to be non-hereditary. More than 90% of children with Wilms tumor present with a palpable lumbar and abdominal mass with a smooth surface and abdominal bulge, and about 30% present with hematuria or abdominal pain, with some presenting with hypertension. It can be complicated by genitourinary malformations, eccentric hypertrophy, Bechwith-wiedmann syndrome, aniridia, etc. The most important prognostic factors affecting Wilms’ tumor in children include the tissue type of the tumor (good or poor prognosis) and the clinical stage of the tumor, as well as whether the child’s family cooperates with further chemotherapy and radiotherapy treatment after surgery. During the surgery, the tumor was removed carefully and completely, so as not to break the huge tumor, and attention was paid to the pathology of the large paravalvular lymph nodes, and the ipsilateral adrenal gland should be removed from the tumor of the upper pole of the kidney. Through careful and responsible treatment by professional physicians, the chances of recurrence and metastasis in children with Wilms tumor after surgery are reduced. The future direction of Wilms tumor treatment is to reduce the occurrence of complications in the treatment of low-risk patients and to improve the long-term survival rate of high-risk patients. Relying on the strong strength of pediatric medicine and surgery, Xinhua Hospital has achieved good results in the treatment of Wilms tumor. It is our endeavor to obtain the maximum treatment result with the least loss of treatment.