Mayer-Rokitansky-Kuester syndrome: a sexual birth defect characterized by impaired differentiation of the Müllerian ducts so that the uterus is rudimentary and cordlike. The deep part of the vagina is absent and the outer part is shallow or in the form of a dimple. The fallopian tubes may be defective, and there may be other sporadic congenital anomalies. The ovaries are normal and induce normal femininizing puberty, except for lack of menstruation secondary to the defective uterus. Psychosexual differentiation is as a female.

Dictionary of Sexology Project: Main Index