To determine the role of lymph node dissection (LND) in the treatment of upper tract transitional cell carcinoma (UTTCC), as the role of LND along with nephroureterectomy in treating UTTCC is unclear and several retrospective studies have been published on this topic with conflicting results. Methods: The Medical Literature Analysis and Retrieval System Online (MEDLINE), the Excerpta Medica dataBASE (EMBASE), Cochrane Central Register of Controlled Trials database (CENTRAL), Cumulative Index to Nursing and Allied Health Literature (CINAHL), Clinicaltrials. gov, Google Scholar, and individual urological journals, were searched for all studies investigating the role of LND in the treatment of UTTCC. Of the studies identified, those that met inclusion criteria were included in this review. Results: In all, 27 studies were included in this review, with 9303 patients who underwent LND. No randomised controlled trials (RCTs) were identified. Tumours were located in the renal pelvis in 62% of patients, in the ureter in 35.5%, and multifocal in 2.3%. In total: 77.1% were LN-negative and 22.9% had LN metastasis. For all patients undergoing LND, the 5-year recurrence-free survival (RFS) and cancer-specific survival (CSS) rates were 27-65.4% and 32.3-95%, respectively. For patients who underwent a LND in accordance with a standardised anatomical template, the 5-year RFS and CSS rates were 84.3-93% and 83.5-94%, respectively. Conclusion: LND may provide a survival benefit in patients undergoing nephroureterectomy for UTTCC, particularly if following a standardised anatomical template and in those patients with muscle-invasive disease; however, a prospective RCT is required to confirm this.