Mayer Rokitansky Kuster Hauser (MRKH) syndrome is a congenital disorder involving reproductive, genitourinary, bone, and cardiac malformation. The incidence is 1 in 4000–5000 females livebirths. The phenotype is female 46 XX karyotype, normal secondary sexual characteristics, and normal functional ovaries. The occurrence of leiomyoma in uterine remnant in MRKH syndrome is a very rare case, even though several cases have been reported. The diagnosis and management approach, in this case, is quite challenging. Here, we report a 38 years old female who represents multiple leiomyomas on the rudimentary uterus, then we did laparoscopic removal of the fibroids and adjacent rudimentary uterus.
- Case report
- Mayer Rokitansky Kuster Hauser (MRKH) syndrome
- Multiple leiomyomas
- Rudimentary uterus