Transitional cell carcinoma of the upper urinary tract is relatively uncommon, representing around 5% of urothelial cancers, and up to 10% renal cancers. Population data suggest that its incidence is gradually increasing, particularly ureteric transitional cell carcinoma. The Balkan countries and the south west of Taiwan have the highest rates of disease. Most tumors involve the renal pelvis, and present with high-grade disease. Up to 50% are muscle invasive (at least) at presentation. However, more cases are now being diagnosed at an earlier stage. Patients tend to be elderly, with men being twice as likely to develop the disease. Smoking is the most common risk factor, but aromatic hydrocarbons are still a factor in industrialized countries. Nearly half of patients will develop metachronous bladder transitional cell carcinoma, most occurring in the first few years. Prognosis depends on a number of factors, including initial stage and grade, lymphovascular invasion, and presence of hydronephrosis.