(A) Intravenous pyelogram of BBS kidney showing pyelocalyceal dilatation, calyceal clubbing, and pericalyceal cysts. (B) ADPKD kidneys typically have numerous cysts of variable sizes, with even distribution throughout the renal cortex and medulla. (C) ARPKD kidneys retain a reniform configuration with radiating, fusiform nonobstructive dilatations of the collecting ducts extending from the medulla to the cortex. (D) In NPH — as in JBTS, Senior-Loken Syndrome, and Jeune syndrome — the kidney exhibits cysts arising from the corticomedullary junction. (E) MKS kidneys manifest cystic dysplastic changes. Images reproduced with permission from Radiology (125) (A), Journal of the American Society of Nephrology (113) (D), and Advances in Anatomic Pathology (126) (B, C, and E). Scale bar: 1 cm.