A 72-year-old female patient presented to our clinic with the chief complaint of gross hematuria and urinary frequency on September 6, 1990. Cystoscopic examination revealed a thumb's head size nonpapillary tumor. The tumor was located adjacent to the orifice of the left ureter. Histological findings of the tumor by transurethral resection (TUR) indicated transitional cell carcinoma with partial signet ring cell carcinoma. No other malignant findings in any other organs including the gastrointestinal tract were noted. Total cystectomy was performed and Indiana pouch was constructed. Histopathological staging was pT1 N0 M0. The patient died of multiple metastasis of the signet ring cell carcinoma on June 22, 1992. This is the thirty-second case of signet ring cell carcinoma of urinary bladder reported in the medical literature in Japan. We investigated 19 alive or unknown cases as follow up and briefly discussed the treatment and outcome of the primary signet ring cell carcinoma of the urinary bladder. The outcome appeared to be somewhat better than previous reports. Total cystectomies were performed in 18 of the 32 cases (56.3%). As noted in past reports, the treatment of our patient consisted of total cystectomy. Twenty-two patients died of signet ring cell carcinoma. Recurrence to the pelvic area was observed in 18 of the 22 (81.8%) patients who died. Because of this high rate of recurrence, we recommend a thorough assessment of the pelvic area of the patients diagnosed with signet ring cell carcinoma of urinary bladder.