Purpose: Plasmacytoid carcinoma of the urinary bladder or plasmacytoid urothelial carcinoma (PUC) is a rare and only recently described histological variant of transitional cell carcinoma (TCC). We herein report the clinical and histopathological features for a new case of PUC. By combining with those reported cases, we intend to define the characteristics of PUC and to provide a therapeutic and prognostic guidance for this disease. Materials and Methods: The index case at our institution was a patient with complaint of lower abdominal pain but without any urological symptoms. The patient underwent radical cystectomy, and the representative sections of tumor were submitted for immunohistochemical analysis. The data for this patient were collected from clinical charts, histological review and follow-up studies. We also performed an extensive literature review of PUC including clinical presentation, pathological features, therapy and prognosis. Results: Clinically, patients with PUC are associated with nonspecific abdominal pain but absent of hematuria. Cystoscopy analysis revealed that PUC is manifested by the coarse and indurated mucosal fold. Macroscopic studies demonstrated an ulcerated firm mass which was present in the left lateral wall of the bladder. Histologically, PUC appeared to be dyscohesive, plasmacytoid cells with eccentric nuclei and abundant eosinophilic cytoplasm with characteristics of plasmacytoid morphology. The tumor cells are negative for E-cadherin, but positive for CD138 expression. This particular patient died 3 months after the radical cystectomy and one course of adjuvant chemotherapy. Literature review revealed that most PUC cases showed similar clinical and pathological features along with poor prognosis. Conclusions: PUC is a rare tumor associated with poor prognosis due to its advanced clinical stage upon its diagnosis. The delayed diagnosis is mainly due to the late occurrence of hematuria and absence of papulary mucosal surface at cystoscopy. Diagnosis can be achieved based on its typical histological features, clinical history and immunohistochemical results. Other than radical cystectomy, postoperative adjuvant treatment could be a good approach to prolong the survival time of PUC patients.