Congenital vesicovaginal fistula is a very rare entity, the etiology of whi
ch has not been clearly elucidated because pathologic features have not bee
n mentioned in previous reports. The case of a 4-year-old girl having incon
tinence resulting from a congenital vesicovaginal fistula joining with the
left ectopic ureter from the hypoplastic kidney is described. This is thoug
ht to be the first presentation of congenital vesicovaginal fistula joining
with ectopic ureter. A microscopic examination revealed the fistula consis
ting of transitional cell epithelium, suggesting an abnormal fusion of the
ureteral bud and caudal end of the mullerian duct with the urogenital sinus
.