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The etiology is a dysfunction or stenosis of the distal ureter. Most often PMU remains asymptomatic with spontaneous resolution allowing for non-operative management. Nevertheless, in selective cases such as the development of febrile urinary tract infections, worsening of the ureteral dilatation, or reduction in relative renal function, surgery should be considered.<\/jats:p><jats:p><jats:italic>Conclusion<\/jats:italic>: Ureteral replantation with excision of the dysfunctional ureteral segment and often ureteral tapering is the gold-standard procedure for PMU, although endoscopic treatment has been shown to have a fair success rate in many studies. In this review, we discuss the natural history, follow-up, and treatment of PMU.\n<jats:table-wrap><jats:table><jats:tbody>\n                    <jats:tr>\n                      <jats:td align=\"left\" colspan=\"2\"><jats:bold>What is Known:<\/jats:bold><jats:italic>\u2022 PMU is the result of an atonic or stenotic segment of the distal ureter, resulting in congenital dilation of the ureter, and is frequently diagnosed on routine antenatal ultrasound.<\/jats:italic><\/jats:td>\n                    <\/jats:tr>\n                    <jats:tr>\n                      <jats:td align=\"left\" colspan=\"2\"><jats:bold>What is New:<\/jats:bold><jats:italic>\u2022 Most often, PMU remains asymptomatic and clinically stable, allowing for non-operative management.<\/jats:italic><jats:italic>\u2022 Nevertheless, since symptoms can appear even after years of observation, long-term ultrasound follow-up is recommended, even up to young adulthood, if hydroureteronephrosis persists.<\/jats:italic><jats:italic>\u2022 Ureteral replantation is the gold standard in case surgery is needed. In selected cases, however, HPBD could be a reasonable alternative.<\/jats:italic><\/jats:td>\n                    <\/jats:tr>\n                  <\/jats:tbody><\/jats:table><\/jats:table-wrap><\/jats:p>","DOI":"10.1007\/s00431-024-05494-7","type":"journal-article","created":{"date-parts":[[2024,3,5]],"date-time":"2024-03-05T10:02:23Z","timestamp":1709632943000},"page":"2029-2036","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Primary non-refluxing megaureter: Natural history, follow-up and treatment"],"prefix":"10.1007","volume":"183","author":[{"given":"Giulio","family":"Rivetti","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierluigi","family":"Marzuillo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Guarino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Di Sessa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"La Manna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony A.","family":"Caldamone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfonso","family":"Papparella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmine","family":"Noviello","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,3,5]]},"reference":[{"key":"5494_CR1","doi-asserted-by":"publisher","first-page":"126","DOI":"10.3389\/FPED.2019.00126","volume":"7","author":"A Dekirmendjian","year":"2019","unstructured":"Dekirmendjian A, Braga LH (2019) Primary non-refluxing megaureter: Analysis of risk factors for spontaneous resolution and surgical intervention. 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