Upper tract urothelial carcinoma (UTUC) is a rare malignancy that is frequently diagnosed at an advanced stage. The diagnostic methods include CT urography, cytology of the upper urinary tract, and ureterorenoscopy (URS). Treatment decisions are guided by risk stratification into low- and high-risk UTUC. In cases of high-risk UTUC, radical nephroureterectomy with bladder cuff excision is considered the surgical gold standard. However, organ-sparing procedures may also be considered in selected cases. Due to the significant reduction in kidney function following RNU and recent prospective data showing favourable radiological and pathological remission rates, the importance of neoadjuvant chemotherapy is being increasingly discussed. For tumours classified as pT2 to pT4 or those with positive lymph node involvement (pN+), adjuvant platinum-based combination chemotherapy is recommended, provided that neoadjuvant chemotherapy has not already been administered. Adjuvant immunotherapy with nivolumab demonstrated no significant therapeutic benefit in the UTUC cohort compared with its use for the treatment of bladder cancer. It should only be considered for patients with tumours ≥pT3 and/or pN+ (or≥ypT2 and/or ypN+ after neoadjuvant chemotherapy) who are either ineligible for or decline platinum-based combination chemotherapy.