Mullerian agenesis is an important cause of primary amenorrhea with normal thelarche and adrenarche. Hormonal studies including gonadotropins, estradiol, progesterone, thyroid hormones, prolactin and testosterone levels are normal. Management of these cases requires attention to the psychological aspect, sexual and reproductive issues. Creation of a vagina can be achieved either by use of dilatators or surgery. For reproduction, the patient benefits from advances in reproductive technology, and in the future uterine transplantation.
