UNLABELLED: What's known on the subject? and What does the study add? Endoscopic management of upper tract urothelial carcinoma (UTUC) using either ureteroscopy and laser ablation, or percutaneous resection, is a management option for treating selected low-grade tumours with favourable characteristics. However, the evidence base for such practice is relatively weak, as the reported experience is mainly limited to small case series (level of evidence 4), or non-randomised comparative studies that are unmatched for tumour stage (level of evidence 3b), with variability of follow-up duration and reported outcome measures. The present systematic review comprehensively reviews the outcomes of all studies of endoscopic management of UTUC, including the role of topical adjuvant therapy. It establishes for the first time a structured reference for endoscopic management of UTUC, and is a foundation for further clinical studies. OBJECTIVE: To systematically review the oncological outcomes of upper tract urothelial carcinoma (UTUC) treated with ureteroscopic and percutaneous management. The standard treatment of UTUC is radical nephroureterectomy (RNU). However, over the last two decades several institutions have treated UTUC endoscopically, either via ureteroscopic ablation or percutaneous nephroscopic resection of tumour (PNRT), for both imperative and elective indications. METHODS: For evidence acquisition the Pubmed database was searched for English language publications in December 2011 using the following terms: upper tract (UT) transitional cell carcinoma (TCC), upper tract TCC, UTTCC, upper tract urothelial cell carcinoma, upper tract urothelial carcinoma, UTUC, endoscopic management, ureteroscopic management, laser ablation, percutaneous management, PNRT, conservative management, ureteroscopic biopsy, biopsy, BCG, mitomycin C, topical therapy. RESULTS: There are no randomised trials comparing endoscopic management with RNU. Most published studies were retrospective case series (and database reviews), or unmatched comparative studies. There was strong selection bias for favourable tumour characteristics in many endoscopically treated groups. There was variation in medical comorbidity and indication for treatment across different study groups. The biopsy verification of underlying UTUC pathology was inconsistent. The follow-up in most studies was limited, typically to a mean 3 years. CONCLUSIONS: There is a high rate of UT recurrence with endoscopically managed UTUC, and a grade-related risk of tumour progression and disease-specific mortality. Overall, renal preservation may be high with ≈20% of patients proceeding eventually to RNU. For highly selected Grade 1 (or low-grade) disease managed in experienced centres, 5-year disease-specific survival (DSS) may be equivalent to RNU, although the small study groups and short follow-ups preclude comments on less favourable Grade 1 (or low-grade) tumour characteristics, or DSS, in the longer-term. For Grade 3 (or high-grade) disease, DSS outcomes are poor and endoscopic management should only be considered for compelling imperative indications in the context of the patient's overall life expectancy and competing comorbidity.