Background: Persistent urogenital sinus is one of the rare urogenital anomalies, which commonly presents as hydrometrocolpos. Fetal urinary ascites as a presentation of persistent urogenital sinus is extremely rare.
Case report: We report on a preterm infant with antenatal diagnosis of hydrometrocolpos and massive urinary ascites secondary to urogenital sinus without any bladder or renal abnormalities.
Conclusion: This case report emphasizes the importance of maintaining a high index of suspicion in the diagnosis of persistent urogenital sinus especially in infants presenting with urinary ascites along with hydrometrocolpos.
Keywords: Ascites; hydrometrocolpos; preterm; urogenital sinus.