Cystic partially differentiated nephroblastoma (CPDN) is among the rare kidney tumors of childhood. Here, a case of CPDN in a baby who was operated with the diagnosis of multicystic kidney is reported. Clinical and imaging studies have a limited role in the differential diagnosis and diagnosis of this entity. A careful histopathological study is required to distinguish between CPDN and other types of multicystic kidneys. Because of the rare potential for aggressive behavior of this tumor, nephrectomy is the treatment of choice alone.
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