Plasmacytoid urothelial carcinoma is an extremely rare pathological finding. We report our experience of one case. A 60 year old male with hematuria of two years evolution, with frequency and dysuria. A tumor was found and he received surgical treatment by TURB at first. The pathology result was a plasmacytoid urothelial carcinoma. Subsequently a radical cystectomy with urinary diversion was performed. The patient received follow-up until his death.