Mostyn Embrey syndrome

Mostyn Embrey syndrome is a rare disorder associated with unilateral renal agenesis and malformations of the female reproductive tract. Delayed diagnosis is associated with serious diagnostic difficulties that may lead to inappropriate management including harmful surgery. The aim of this paper is to describe the first case of this rare syndrome in Iraq which was originally reported in 2016, and to provide an overview of the previously reported cases. Less than 500 cases of the classical Mostyn Embrey syndrome (associated with triad of uterus didelphys. Hemi-vaginal obstruction and ipsilateral renal agenesis) have been reported in the literature during the previous 100 years, and 560 cases of the classical Mostyn Embrey syndrome and its variants have reported in the literature during the previous 100 years. The first case of Mostyn Embrey syndrome is Iraq was the case number 561, and the second in Ethnic Arab. This case was the first case of Mostyn Embrey syndrome to have family history of incomplete variant syndrome in a cousin.