Survivin belongs to a family of proteins, which serve as inhibitors of apoptosis. Survivin inhibits apoptosis by blocking activation of effector caspases in both extrinsic and intrinsic pathways of apoptosis. Expression of survivin has been demonstrated in several malignant neoplasms and is generally associated with adverse prognosis. In the case of bladder cancer, survivin is expressed in the neoplastic epithelium but not in the uninvolved mucosa. Several studies on bladder cancer have indicated that there may be a relationship between survivin expression and ultimate behavior of the carcinoma, although the exact nature of this relationship is still not fully understood, because the results of some of these studies seem to be contradictory. As survivin is differentially expressed in bladder cancer and not in the normal urothelium, several studies have demonstrated efficacy of urine testing of survivin as a diagnostic tool for an early detection of bladder cancer. Survivin has also been suggested as a suitable target for developing specific therapy for local treatment of bladder cancer with encouraging initial results. Thus, survivin is a potentially significant protein with a crucial role in the diagnosis, prognosis, and treatment of bladder cancer.