Mayer-Rokitansky-Kuster-Hauser Syndrome

Abstract

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is a form of Mullerian agenesis and is a cause of primary amenorrhea in females. It is characterized by congenital aplasia of uterus and upper two third of vagina showing normal development of secondary sexual characters and normal 46XX karyotype. Here we describe a case of a young female presenting with primary amenorrhea that was diagnosed as a case of Type 2 MRKH syndrome.