Vagina atresia is a rare congenital genital tract anomaly caused by abnormal development of the urogenital sinus and the caudal part of the Müllerian ducts during embryonic development. It is characterized by partial or complete absence of the vagina, with usually well-developed and functional uterus. The incidence of congenital absence of vagina is 1/4000 to 1/5000, of which only 7–8% were vaginal atresia associated with normal uterus. Vaginal atresia is one of the obstructive genital tract malformations causing typical symptoms of primary amenorrhea, cyclical lower abdominal pain, and pelvic mass. Conservative surgical corrections are fundamental approaches relieving the symptoms and preserving the patients’ fertility reservation.