Upper tract urothelial carcinoma (UTUC) is a rare cancer with a low annual incidence (1.2 cases/100,000 population per year) and accounts for 5–10% of urothelial carcinomas. More than 90% of upper tract tumours are urothelial carcinomas, and their grade classification is based on bladder cancer classification; however, the natural history and aetiology of UTUC differ from those of bladder cancer. Several risk factors are known; some can be avoided (tobacco, occupational exposition, acid aristolochic) and some are hereditary (hereditary non-polyposis colorectal carcinoma). In the case of suspicion of UTUC, urinary cytology, computed-tomography urography (CTU) and cystoscopy are mandatory. CTU is composed of four phases and is the gold-standard procedure to detect UTUC; however, it does not assess tumour stage. Flexible ureteroscopy and in situ cytology are performed either to confirm suspicious cases or before conservative treatment. Flexible ureteroscopy should be performed without an access sheath and with a low intra-renal pressure in order to avoid tumour spilling. Narrow-binding imaging and photodynamic diagnosis enhances the visualization of UTUC during surgery.