A 70-year-old Japanese man complained of macrohematuria. He was diagnosed with high grade urothelial carcinoma (UC), G3, and underwent transurethral resection of bladder tumor (TUR-BT) twice. UC containing a chordoid component was found in the TUR-BT tissue samples. Total cystectomy combined with nephroureterectomy was performed because of deep muscular invasion into the bladder, in addition to intensive ureteral and prostatic invasion. Histopathological findings from the bladder tumor revealed an invasive UC with an adenocarcinoma component, combined with peculiar chordoid features associated with abundant myxoid matrix. The chordoid component metastasized to the right kidney, invading the prostate and right ureter. Chordoid component was detected in approximately 50% in total tumor extent Immunohistochemical analysis indicated that, although the chordoid component showed similar staining results to those of chordoma (namely, it was positive for CAM5.2 and cytokeratin 19 and focally positive for epithelial membrane antigen, carcinoembryonic antigen, and S100 protein), the UC and chordoid components were both negative for vimentin. In contrast, both chordoma and extraskeletal myxoid chondrosarcoma demonstrate positivity for vimentin, which is histologically similar to the present case. The patient had come to see us, before 11 months. A follow-up assessment showed no recurrence or distant metastasis 8 months after twice TUR-BT and five months since total cystectomy. But unfortunately, the patient dropped out from our follow-up data, because he did not come to our hospital. Thus, pathologists and clinicians must consider that a chordoid component may represent the malignant characteristic of UC.