Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital condition in which a genetic female is born with vaginal agenesis and a rudimentary to absent uterus. This condition affects a woman's ability to menstruate, to engage in penile-vaginal intercourse, and to bear children. Much has been published about how best to create a neovagina in women with MRKH, but little has been written about the psychological impact of MRKH and quality of life outcomes for women with the condition. A review of the extant literature published from 1955 to 2007 supports that (1) surgical or non-surgical creation of a neovagina alone does not ensure a successful psychological outcome, (2) psychological support at critical times can be helpful, and (3) how professionals use language to discuss the condition may positively or negatively influence a female's experience of MRKH. This article discusses the implications that existing knowledge has on future research and on clinical practice. Understanding how women with MRKH cope with and adjust to the condition will help healthcare professionals provide optimal care.