Congenital anomalies of the upper urinary tract are prevalent in both children and adults, categorized as anomalies of the kidney and anomalies of the renal pelvis and ureter. Anomalies of the kidney encompass renal agenesis and hypoplasia, renal dysplasia, renal ectopia and anomalies of fusion, anomalies of rotation, and anomalies of the renal calyces. Anomalies of the renal pelvis and ureter include congenital ureteropelvic junction obstruction, congenital megaureter, circumcaval ureter, duplication of the collecting system, ureterocele, ectopic ureteral insertion, and vesicoureteral reflux. Since the majority of these disorders originate from a series of abnormal embryological processes, it is important to understand the multiple associated abnormalities accompanying congenital anomalies in the upper urinary tract. Multimodality imaging, such as computed tomography (CT), magnetic resonance imaging (MRI), ultrasonography (US), and intravenous urography (IVU), are essential for evaluating the spectrum of the disorder. This chapter presents diverse imaging findings related to the congenital anomalies of the upper urinary tract.