Wilms tumor, or nephroblastoma, is an infrequent form of kidney cancer predominantly observed in pediatric patients. Named after Dr. Max Wilms, who first described it in 1899, this tumor typically originates in the cells of the developing kidneys during fetal development. Wilms tumor is most commonly diagnosed in children aged 3 to 4 years, and it rarely occurs in older children or adults. The exact cause of Wilms tumor is not well understood, but certain genetic factors and abnormalities appear to contribute to its development. Symptoms of Wilms tumor may include abdominal swelling or a mass, abdominal pain, blood in the urine, fever, and hypertension. Diagnosis often involves imaging studies such as ultrasound, CT scans, and MRI, as well as a biopsy to confirm the presence of cancerous cells. Treatment for Wilms tumor typically involves a combination of surgery, chemotherapy, and sometimes radiation therapy. The goal is to remove the tumor while preserving as much healthy kidney tissue as possible. The overall prognosis for Wilms tumor is generally favorable, with a high rate of cure, especially when detected and treated in its early stages. Regular follow-up care is essential to monitor for any signs of recurrence and to address potential long-term effects of the treatment. Despite its challenges, advancements in medical research and treatment protocols have significantly improved the outcomes for children diagnosed with Wilms tumor. A multidisciplinary approach involving pediatric oncologists, surgeons, and other specialists is crucial in providing comprehensive care for affected individuals.