We report a successful case of persistent urogenital sinus associated with a duplicated nonsyndromic form of hydrometrocolpos and ascites diagnosed prenatally. Though urogenital malformations are extremely rare and variable in presentation, the conjugation of those anomalies in a newborn is reported here for the first time. Prenatal ultrasound diagnosis was suspected at 25 weeks’ gestation and MRI imaging supported the diagnosis. Periodic ultrasound surveillance was conducted until birth at 35 weeks’ gestation by cesarean section. The presumptive diagnosis was confirmed after birth. One month later, the newborn underwent reconstructive surgical intervention with good outcome.