A case with an unusual variation of urogenital sinus abnormalities is reported. A newborn was referred with abdominal distention and abnormal perineum with anus. Colostomy and cystostomy were performed at birth. Endoscopy and fluoroscopy showed urogenital sinus malformation with vesicovaginal fistula, rectovesical fistula and anorectal stenosis. I.V.P. showed left hydronephrosis. It seemed that this is a rare case of urogenital sinus abnormality associated with vesicovaginal fistula, rectovesical fistula and anorectal stenosis, although it was extremely difficult to make a complete embryological explanation for this case. At age 2 years a reconstructive operation was performed. It included abdominal perineal pullthrough of the sigmoid colon, separation of the vagina from the bladder and joining the vagina to the perineum with a segment of sigmoid colon. Urogenital sinus was preserved as a urethra. Postoperatively the infant could not empty her bladder spontaneously and intermittent catheterization has being continued by the mother. Now at age 4 years, bowel control is almost satisfactory.
