Congenital abnormalities of the kidneys and urinary tract (CAKUT) encompass a spectrum of pathology ranging in severity from incidental findings to marked deviations of anatomic form and function. The imaging appearance of CAKUT generally reflects a deviation from normal embryologic development. Common and uncommon congenital abnormalities of the kidneys, ureters, bladder, and urethra are reviewed with an emphasis on the imaging appearance and embryologic origin so that they may be promptly identified and treated when encountered in clinical practice.