In 2004, the WHO recognized the plasmacytoid subtype as a distinct variant of vesical malignancy. We present a case of plasmacytoid urothelial carcinoma of the bladder treated with radical surgery and adjuvant chemotherapy, thus achieving long-term survival. A 70-year-old woman presented with persistent dysuria and underwent cystoscopy which revealed the presence of diffuse deformity, involving the right lateral vesical wall. Histology revealed the presence of muscle-invasive urothelial carcinoma of the plasmacytoid variant. The patient subsequently underwent radical cystectomy and orthotopic ileal neobladder substitution, as well as adjuvant chemotherapy. At 36 months of follow-up, the patient is free of local recurrence and metastases, while her voiding function is well preserved. Bladder plasmacytoid urothelial carcinoma is considered a rare tumor, with unique microscopic and immunohistochemical features. The ideal therapeutic approach is debatable, but the combination of radical surgery and chemotherapy should constitute the mainstay of management.