Unilateral renal cystic disease in a 15-year-old female: A left huge kidney associated with significant morbidity

Unilateral renal cystic disease (URCD) is a rare nonfamilial, nonprogressive disorder of one kidney and is not related with autosomal dominant polycystic kidney disease. The present case is a report of a 15-year-old female who had a huge multicystic mass involving the lower and middle part of the left kidney with normal minimal residual renal parenchyma at the upper pole. All the laboratory tests were within normal limits. The right kidney was entirely normal. Left nephrectomy was performed because of a significant morbidity. Here, the clinic, radiologic and pathologic findings are described and their differential diagnosis is discussed.