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Caroli's syndrome associated with medullary sponge kidney and nephrocalcinosis.

Congenital non-obstructive multifocal dilatation of the intrahepatic bile ducts (Caroli's syndrome [1]) is a rare condition. Unlike true hepatic cysts, the cystoid ducts contain bile. Caroli's syndrome can be present in a simple form with monolobar (often left lobe) or diffuse bile duct dilatation or in an associated complex form with additional extrahepatic bile duct dilatations, congenital hepatic fibrosis, portal hypertension and cystic malformation of the pancreas, spleen or kidneys. Medullary sponge kidney, which was first described as a pathological entity in 1948 [2], consists of an ectasia of the terminal collecting ducts in the pericalyceal region of the renal pyramids with formation of small and large bilateral diffuse cysts. It is a benign condition with an excellent long-term renal prognosis. This case report presents the clinical and radiological findings of a patient with Caroli's syndrome and unilateral preponderance of nephrocalcinosis due to medullary sponge kidney.