Background: The Vagina is a composite structure formed partly from the mullerian duct and partly from urogential sinus. Most of the patients with mullerian agenesis have small rudimentary uterus without any endometrial cavity, 7% - 8% may have functioning uterus. Case: A 15 years old girl admitted with primary amenorrhea, cyclical abdominal pain, hematometra and complete vaginal agenesis. Surgical correction is the mainstay of treatment in patients with functioning uterus and absent vagina. Vaginoplasty was done to reconstruct a neovagina using amnion graft and establish uterovaginal continuity. In this case we have used only perineal approach to dissect the neovagina and identify the cervix with formation of a patent drainage tract and adequate vaginal length of 9 cm. Conclusion: In rare cases of obstructed functioning uterus by complete vaginal agenesis, vaginoplasty effectively restores anatomy and function by an overall minimal operative morbidity. Human amnion provides excellent results in neovaginal reconstruction.
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