Background: Persistent urogenital sinus is a rare cloacal anomaly in which the urinary and genital tracts cannot be diferentiated during embryonic development. However, the presence of concomitant recto-urogenital sinus or rectovaginal fstula is much rarer. In the literature, only two cases with accompanying fstula have been presented so far. Case presentation: We present the diagnosis and treatment management of a persistent urogenital sinus case with recto-vaginal fstula. We also aimed to reveal the diference between our patient, who is the 3rd case in the literature, and the other two and the described cloacal anomalies. By performing total urogenital sinus mobilization and fstula repair, the three systems were separated from each other, and both a functional and cosmetic appearance was obtained. Conclusion: It should be kept in mind that there may be diferent variants other than the defned cloacal malformations. In patients with cloacal malformation, cystoscopy and rec