Abstract

The Mayer-Rokitansky-Kuster-Hauster (MRKH) Syndrome is a rare congenital mullerian duct agenesis with various degrees of clinical manifestations ranging from primary amenorrhea to complete absence of vagina, even though they may have secondary female sexual characteristics [1-3]. the incidence varies from region to region with different researchers reporting various figures from 1 in 4,000 to 1 in 10,000 female life births [4-6] some of the patients may present with a normal uterus, rudimentary bi-cornuate, with or without lumen or it may totally be absent as it occurs in our patient and most of the patients being 46XX have ovaries, they may have blind end or completely absent vagina [7].