Abstract
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital anomaly characterized as aplasia or hypoplasia of uterus and vagina in women with normal development of secondary sex characteristics. It affects 1 in 4000-5000 female births. Women with this syndrome present with primary amenorrhoea. MRKH syndrome may be associated with renal, skeletal, cardiac and auditory anomalies. Women with MRKH syndrome may develop leiomyoma from a rudimentary uterus, though very rare. Initial investigation in women having MRKH syndrome with leiomyoma is ultrasonography (USG). However, CT and MRI are more accurate to evaluate the pelvic anatomy and pathologies.