DOI: 10.36347/sjmcr.2017.v05i11.032

Pages: 795-797

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital Müllerian duct aplasia of the uterus and the upper part (2/3) of the vagina in women showing normal development of secondary sexual characteristics and a normal 46XX karyotype. Situs inversus is a rare congenital anomaly characterized by the transposition of the abdominal organs, viscera and vasculature. When associated with dextrocardia it is known as situs inversus totalis. Here we report a case of 35 year old lady presented with primary amenorrhea and absence of upper vaginal orifice with normal secondary sexual characteristics. After laparoscopy, hormonal assay and karyotyping she was diagnosed as MRKH syndrome. On Ultrasound abdomen we found all the abdominal viscera were located just opposite, associated with right sided renal agenesis and on chest x-ray there was dextrocardia i.e. situs inversus totalis.