Mayer-Rokitansky-Kuster-Hauser syndrome Type 2 or MURCS co-occurrence with Benign Ovarian Tumor – a tremendous case report

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS) is a congenital condition characterized by aplasia of the vaginawith or without concurrent uterine and/or cervical aplasia.Between the two types ,type 2 or MURCS is a rare formincluding Mullerian agenesis,Renal agenesis and Cervicothoracic somite anomalies.A 17 years old virgin presentedwith feeling of heaviness and lump in abdomen for 6 months with history of primary amenorrhoea till the date anda diagnosed case of MRKH with normal female karyotype 46XX also having unilateral left sided renal agenesis andhistory of multiple surgery for congenital heart disease (TOF), repair of inguinal hernia and laparotomy for pelvicmass with biopsy taking. Evaluation of the patient suggests a tremendous presentation of MURCS association withbenign ovarian tumor. A review of the literature reveals no other cases of MRKH with these tremendous anomalieshaving history of corrected TOF and inguinal hernia as well as with co occurrence of a huge benign ovarian tumor.