The congenital aplasia or severe hypoplasia of mullerian structures is infrequent. However, the features of normal female endocrine function paired with the absence of a functional uterus and vagina makes it a fascinating entity. The diagnosis and work-up in these patients has become very efficient, thanks to the use of imaging, and there are multiple successful procedures for the creation of a neovagina. In recent years, infertility treatment options through in vitro fertilization have also become available as part of the long-term care of these patients.
Keywords: MRKH; mullerian agenesis; neovagina; vaginal agenesis.