Bladder cancer remains a frequent cancer worldwide, and most tumors are diagnosed at localized stages. Urothelial carcinoma (UC) accounts for 90% of bladder cancer cases. Sarcomatoid carcinoma (SaC) of the bladder is a rare variant (0.5% of total bladder cancers) characterized by 2 components based on histology; the epithelial and mesenchymal phenotypes, which can be easily differentiated by immunohistochemistry. SaC has similar epidemiologic features to UC but different behavior, aggressiveness, and prognosis. In this review, we summarize the main differences between UC bladder cancers and SaC subtypes. The therapeutic strategies used in SaC today do not differ much from those used for the urothelial variant. However, there is still no standard treatment—the result of a lack of clinical trials for the sarcomatoid variant. Further multicenter comparative studies are needed to devise a better treatment strategy for patients with this rare histologic tumor subtype.