We present a case of a premature female neonate with a triad of persistent urogenital sinus with urinary ascites, bilateral hydrocolpos in a duplex uterus, and abdominal wall hypoplasia resembling Prune Belly-like syndrome, combined with severe bilateral cystic dysplastic kidneys and complex urinary obstruction. A female infant was born at 343/7 weeks' gestation via cesarean section due to prenatal detection of hydrops fetalis and massive urinary ascites. Prenatal ultrasound had shown oligohydramnios, ascites, megacystis, and hydrocolpos. Postnatally, urinary ascites, a persistent urogenital sinus, severe upper urinary tract dilation, bilateral dysplastic kidneys, and an obstructive vaginal septum were confirmed. Management included staged urinary drainage, vaginal septum incision, intensive respiratory and renal support, and multidisciplinary care. This unique combination of anomalies presents significant diagnostic and therapeutic challenges. Early recognition and individualized multidisciplinary management are essential to improve postnatal outcomes and guide long-term planning in such cases. E.G.: Conceptualization, data curation, investigation, methodology, writing–original draft, writing–review and editing. J.H.: Conceptualization, data curation, investigation, methodology, supervision, writing–original draft, writing–review and editing. H.C.S.: Conceptualization, investigation, methodology, writing–review and editing. K.W.: Investigation, supervision, writing–review and editing. K.R.: Investigation, supervision, writing–review and editing. C.T.: Conceptualization, data curation, investigation, methodology, supervision, writing–review and editing. The case did not require review by the Ethics committee of the Hamburg Medical Association (ÄKHH) Institutional Review Board (2025-300599-WF). Received: 19 May 2025 Accepted: 04 February 2026 Article published online: 24 February 2026 © 2026. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/) Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany