Upper tract urothelial carcinoma (UTUC) accounts for approximately 5% of all urothelial carcinoma. There are many risk factors for UTUC, including environmental and genetic risk factors, some of which are in common with bladder cancer. The gold standard surgical management of UTUC is radical nephroureterectomy (RNU) with excision of bladder cuff, which is increasingly being performed laparoscopically or robotically with various methods used for the distal ureter. There are increasing numbers of patients being treated endoscopically, with excellent oncological outcomes in low-grade disease. The use of topical BCG and chemotherapy agents has been extrapolated from bladder cancer and may be an adjunct to endoscopic management in those patients in whom it is imperative to avoid RNU.