Upper tract urothelial carcinoma involving the renal pelvis and ureter is a relatively uncommon urologic malignancy and often associated with a poor prognosis. Strategies for surgical management continue to evolve with controversy in many respects. The purpose of this chapter is to provide a contemporary review of these strategies in terms of diagnosis and endoscopic and surgical treatment. Patients who are at the highest risk of developing upper tract urothelial malignancy are those who have been diagnosed with bladder tumors. Following radical cystectomy, the majority of early recurrences can be detected through routine oncologic surveillance. However, long-term recurrences may only be detected following the development of symptoms. Nephroureterectomy has been considered the gold standard in treating upper tract urothelial carcinoma. Laparoscopic nephroureterectomy is now standard; however the ideal management of the bladder cuff remains controversial. In patients in whom nephroureterectomy will lead to dialysis, nephron-sparing treatment options may be preferable. Endoscopic management is effective in this setting, and more recently has been extended to patients with a normal contralateral kidney with low-grade, low-volume tumors. Topical treatment including BCG and mitomycin, and novel agents have also been shown to be effective as an organ-sparing approach. While the advantages of lymphadenectomy have been reported for other genitourinary cancers, the role of lymphadenectomy in upper tract urothelial cancer remains to be determined. Perioperative chemotherapy is commonly offered to patients with advanced upper tract urothelial cancer, and hopefully, more effective therapies and better patient selection will lead to a defined survival benefit with this strategy.