Congenital absence of the vagina

Congenital absence of the vagina is the second leading cause of primary amenorrhea. Three patients had Mayer-Rokitansky-Küster-Hauser syndrome or congenital absence of the vagina. We discuss the clinical, diagnostic, psychosocial, and therapeutic features of these patients and the syndrome. The lack of diagnosis until after puberty of this syndrome in many patients demonstrates the need for more careful genital examination in prepubertal and pubertal girls.