Introduction: Fibroepithelial polyps of the ureter are rare. Cases and small series are reported in the literature. The treatment of choice, outcome and appropriate follow-up regimen remain unclear. Methods: We conducted a systematic literature review of papers reporting fibroepithelial polyps of the ureter in adult patients. Articles published before 1980 were excluded. Results: The search yielded 144 papers, of which 68 met the inclusion criteria. A reference scan from the included 68 yielded an additional 7 new articles. In total, our study included 75 articles (68 + 7). A total of 134 patients were described. Most patients had a single lesion (range: 1-10). The median length of the polyp was 4.0 cm (range: 0.4-17.0). The percentage of polyps resected endoscopically increased from 0% before 1985 to 67% after 2005. Two perioperative complications were reported in 72 procedures (2.8%): a deep venous thrombosis and a case of mesenteric lymphadenopathy. Both of these occurred after open surgery. Follow-up data were available for 57 patients. The median follow-up was 12 months (range: 1-180). Four patients (7.0%) developed recurrent complaints: 2 had urinary stones, 1 had a ureteral stricture and 1 had recurrence of the polyp. Three of these events followed endoscopic resection, and occurred within a year after the procedure. Conclusion: Endoscopic resection of fibroepithelial polyps seems to be safe and effective. It is minimally invasive and should be considered the gold standard where endoscopic expertise is available. We advise follow-up imaging by computed tomographic intravenous urography after 3 months and ultrasound after 1 year to detect late complications.