A 4-day-old female neonate was admitted to Kansai Medical University Hospital because of cyanosis and anuria. Cystogram revealed a dilated urinary bladder. CT scan revealed two cystic regions in the presacral space behind the urinary bladder. We performed subtotal excision of the cyst at the age of 48 days. One of the cysts was the hydrovagina, which was evacuated. The remaining cyst was completely resected at the age of 16 months. The final pathological diagnosis of the presacral cyst was cystic mature teratoma. At the second operation, we could not insert a Foley catheter into the urethra from the external meatus. Cystogram done from the catheter cystostomy and cystoscopy revealed the urethrovaginal fistula. The pathogenesis of the urethrovaginal fistula might be an aquired antenatal anatomical maldevelopment of the urogenital structures caused by the compression of the large presacral teratoma.