Unilateral renal cystic disease in infancy

Unilateral renal cystic disease (URCD) is a non-familial, unilateral, cystic kidney disorder with preserved function of affected kidney. It is a very rare condition, especially in children. The cysts in URCD are histologically indistinguishable from cysts in autosomal dominant polycystic kidney disease (ADPKD). URCD has a mild and non-progressive clinical course. We report a patient with URCD, detected when she was 10-months old, and followed for 5 years so far. She was the third reported patient with URCD under one year of life. Diagnosis was made by ultrasonography, IVU, and radionuclide techniques, besides her family history. Since those methods were sufficient to establish the diagnosis of URCD we could avoid CT and angiography without any significant effect on the diagnostic results.