Distal vaginal aplasia and functional uterus is an uncommon congenital malformation. Reconstructive therapy is controversial. Mental problems may result. The objective in this case was to restore menstruation, and preserve reproductive performance by avoiding hysterectomy, in order to prevent psychological impacts of amenorrhoea and sterility. This case is a 14 years old girl presented with classical features of vaginal obstruction and functional uterus. Physical examination revealed absent vagina and a pelvic mass. The patient and her family were very concerned about menstruation and fertility. Neovagina was constructed successfully using the modified McIndoe vaginoplasty. Eventually abdominal Vagino-neovaginal anastomosis was necessary to restore reproductive tract continuity. The outcome was restoration of menstruation and made future marriage and fertility more actual. This required six surgical interventions over a period of ten months. Associated pelvic endometriosis was observed and right salpingectomy was thought necessary. Although hysterectomy is a shortcut procedure to relieve complications of menstrual retention in vaginal obstruction, it will cause psychological trauma to the patient and her family. Construction of artificial vagina and Vagino-neovaginal anastomosis can be successfully performed to provide the patient an opportunity for conservative management, resulting in menstruation, and some potential for fertility.