Urothelial carcinoma of the upper urinary tract (upper tract urothelial carcinoma, UTUC) is less common than bladder carcinoma with nearly identical risk factors and has a poorer prognosis. The standard diagnostic procedure is imaging of the upper urinary tract by computed tomography urography. In cases of diagnostic uncertainty, a diagnostic ureterorenoscopy with biopsy sampling can be performed in addition to urine cytology. Treatment depends primarily on the stage and grading of the tumor. Depending on the extent and localization, organ-preserving treatment or radical nephroureterectomy is indicated. Perioperative systemic treatment in high-risk UTUC can be performed in both neoadjuvant and adjuvant settings, although the current data on neoadjuvant chemo- and immunotherapy do not yet allow standard application. For metastatic disease, a multimodal treatment approach consisting of cisplatin-based or carboplatin-based chemotherapy, immunotherapy, and treatment with enfortumab vedotin can be considered. Salvage surgery, radiotherapy and metastasectomy are available for rare individual cases.