Lymphoepithelial carcinoma is a relatively common malignancy in the nasopharynx, hut it rarely occurs at other sites. Described herein is the first case of a renal pelvic neoplasm that closely resembled lymphoepithelial carcinoma, with analyses of histology, immunophenotype, in situ hybridization for the Epstein-Barr viral genome, and now cytometric DNA ploidy. The tumor was detected in an 70-year-old Japanese man who presented with hematuria, Histologic examination showed an undifferentiated round or spindle cell carcinoma (cytokeratin 7(+), cytokeratin 20(+), epithelial membrane antigen(+), vimentin(-)) with abundant lymphocytes (predominantly UCHL-1(+) T cells), plasma cells, and macrophages in and around the tumor cell nests. The tumor was limited to the pelvis, with a minute focus of carcinoma in situ. No Epstein-Barr viral genomic sequences were detected by in situ hybridization, The tumor had an aneuploid DNA content. The patient remains well without disease 6 years after surgery and radiotherapy. Recognition of this type of renal pelvis carcinoma is important to avoid misdiagnosis.