Congenital anomalies of the kidney and urinary tract encompass a broad spectrum of developmental conditions that together account for the majority of childhood chronic kidney diseases. Kidney abnormalities are the most commonly diagnosed congenital anomaly in children, and detection of this anomaly is increasing as a result of improved antenatal care and widespread access to more sensitive screening ultrasonography. Most paediatricians will encounter children with congenital kidney anomalies across a wide spectrum of disorders, and a broad understanding of the classification, investigation, and basis of management is important to appropriately direct their care.