The upper urinary tract is the most common human system affected by congenital anomalies. Congenital anomalies of the kidneys and ureters comprise a wide spectrum of disorders ranging from simple variants with no clinical significance to complex anomalies that may lead to severe complications and end-stage renal disease. They may be classified as anomalies of renal form, which are subclassified as structural anomalies (eg, persistent fetal lobulation, hypertrophied column of Bertin, and dromedary hump) and fusion anomalies (eg, horseshoe kidney and pancake kidney); anomalies of renal position (eg, renal malrotation, simple renal ectopia, and crossed renal ectopia) and renal number (eg, renal agenesis and supernumerary kidney); and abnormalities in development of the urinary collecting system (eg, pyelocaliceal diverticulum, megacalycosis, ureteropelvic junction obstruction, duplex collecting system, megaureter, ectopic ureter, and ureterocele). US is usually the first imaging modality used because of its low cost, wide availability, and absence of ionizing radiation. Intravenous urography and voiding cystourethrography are also useful, mainly for characterization of the collecting system and vesicoureteral reflux. However, intravenous urography has been replaced by CT urography and MR urography. These imaging methods not only allow direct visualization of the collecting system but also demonstrate the function of the kidneys, the vascular anatomy, adjacent structures, and complications. Comprehension of congenital anomalies of the upper urinary tract is crucial for an accurate diagnosis and correct management. The authors discuss the spectrum of these anomalies, with emphasis on embryologic development, imaging findings, clinical manifestations, and complications. Online supplemental material is available for this article. ©RSNA, 2021.