In this article, we review available evidence on the treatment of patients with nonurothelial cancer of the bladder. More than 150 published works were reviewed in preparation for this summary. Squamous cell carcinoma and adenocarcinoma are ideally treated with radical cystectomy. High-risk groups for these diseases are defined. Small cell carcinoma should be treated with multimodality therapy, including chemotherapy. Other rarer tumors of the bladder are also discussed.