Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization

We report a rare case of primitive neuroectodermal tumor of the kidney. The diagnosis was confirmed by the immunohistochemical profile and fluorescence in situ hybridization in formalin-fixed, paraffin-embedded tissues. The patient received intensive chemoradiotherapy after radical surgery and remains alive without recurrence 1 year after initial presentation.