Upper tract urothelial carcinoma (UTUC) is an uncommon disease with a prognosis worse than that of primary urothelial carcinoma (UC) of the bladder. Although surgery remains the mainstay of UTUC therapy, there is increasing enthusiasm for combined-modality approaches in both adjuvant and neoadjuvant settings. Nephron-sparing surgical strategies, including partial ureterectomy and purely endoscopic tumor resection, are also increasingly used. Through the development of multi-institutional consortiums, novel treatment algorithms can now be used to evaluate patients more efficiently and thoroughly than in the past. In addition, the genome of UC isolates has recently been sequenced and published, making it possible to identify molecular targets for future therapies. By reviewing the epidemiology, current management strategies, and areas of ongoing research in UTUC, we hope to provide a background of knowledge useful to clinicians managing patients with this challenging disease.