The biggest challenging in managing upper tract urothelial carcionma (UTUC) remains accurate staging of tumors at diagnosis. Given the limited ability of contemporary staging methods, clinical risk stratification models have been developed to assist in providing risk-based treatment recommendations. For example, endoscopic management (e.g., ablation) represents the recommended first line approach for patients with low risk disease. Importantly, follow-up for these patients should include endoscopic re-evaluation. The value of testing patients with UTUC for Lynch syndrome is also critical to recognize. A role for neoadjuvant chemotherapy is being increasing explored for patients classified with high-risk UTUC, while several options exist for adjuvant chemotherapy and immunotherapy for patients with adverse pathology at surgical resection. Continued investigation into prevention of postoperative intravesical recurrences in patients with UTUC is warranted given the frequency of metachronous tumor development in the bladder among these patients.