DOI: 10.36347/sjmcr.2015.v03i11.020

Pages: 1075-1078

Mayer Rokitansky-Kuster Hauser syndrome is a malformation complex comprising of absent vagina and absent or rudimentary uterus. MRKH syndrome may be attributed to initial affection of intermediate mesoderm leading to (by the end of 4th week of fetal life) an alteration of blastomere of cervicothoracic somites and pronephric duct. The latter induces the differentiation of mesonephric and then Wolfian and Mullerian ducts. We present a case of 21 year old married female with primary amenorrhea and normal secondary sexual characteristics with congenital absence of radial artery. The diagnostic investigations demonstrated occurence of Mayer Rokitansky-Kuster Hauser syndrome with unilateral absence of radial artery.