The kidney and urinary system are common sites of congenital anomalies of varying severity. Numerous anomalies are asymptomatic and are diagnosed by prenatal ultrasound or during systematic assessment for other congenital anomalies. Some of those anomalies are diagnosed due to symptoms such as obstruction, infection, or trauma. The spectrum of renal anomalies ranges from completely asymptomatic, which do not interfere with the quality of life, to the ones that are lethal, such as bilateral agenesis of the kidneys. They can be classified into renal parenchymal anomalies, migration abnormalities, and collecting system anomalies. The etiology of renal anomaly is very broad and includes genetics, epigenetics, remains surrounding factors and the inability of urine flow. Of the diagnostic methods, ultrasound is the most important, and so are others like CT, MR, and intravenous urography. Therapy usually depends on the symptoms and other associated anomalies. Surgical procedures usually occur if there is obstruction of urine outflow, if the anomaly causes pain in the patient (nephroptosis), or if there is dysfunctional kidney that is better removed. Since these anomalies are a major cause of chronic kidney disease in children, it is very important to monitor children regularly (measuring blood pressure and proteinuria) and also through the life.