Upper urinary tract urothelial cell carcinoma (UTUC) is a rare entity. The incidence in Germany is approximately 2/100,000 with a ratio between women and men of 1:2.5. Most clinical signs are nonspecific, which is why early diagnosis is rarely successful. Computed tomography urography in combination with diagnostic ureterorenoscopy is currently the gold standard in the diagnostics of UTUC. Regarding surgical treatment, radical nephroureterectomy (RNU) with resection of a bladder cuff remains the method of choice, although the radical approach is developing towards laparoscopic/robotic or endourological procedures with preservation of kidney tissue. Due to the high recurrence rate (22-47%) of urothelial carcinoma inside the bladder, close follow-up after RNU is mandatory.