Nephroblastoma, also known as Wilms' tumor, is a malignant neoplasm that develops from kidney tissue. This disease is considered the most common form of malignant urinary tract tumor in children. Nephroblastoma occurs equally in boys and girls under 5 years of age. Research shows a correlation between maternal age and the likelihood of having a child with nephroblastoma. Wilms' tumor is a congenital anomaly. Wilms' tumor is given in honor of the German surgeon Max Wilms (1867–1918), who in 1899 conducted a comprehensive review of the literature on pediatric kidney tumors and scientifically substantiated the origin of this neoplasm. It should be noted that, although nephroblastoma predominantly occurs in young children, in rare cases it can also occur in older children. Early diagnosis, correct staging of the disease and informed choice of treatment methods play a key role in ensuring the possibility of a full recovery for patients with nephroblastoma.