Cystic partially differentiated nephroblastoma is a rare renal mass in children. Since its clinical
characteristics and radiologic findings are very similar to those of cystic Wilms tumor and cystic
nephroma, the discrimination between these disease categories are known to be difficult and only
possible by histology. A 9-month-old female presented with 2 months history of abdominal
distension. Renal ultrasonography showed a mass with multiple cystic components in the upper
to lower poles of the right kidney and computer tomography showed a well-marginated low
attenuation multilocular cystic mass. Right nephrectomy was performed and cystic partially
differentiated nephroblastoma was diagnosed by pathologic findings. There have been only few
reports in the literature with only one case report in Korea. Here we report a case of cystic
partially differentiated nephroblastoma with some review of the literatures