Background
Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is a rare syndrome affecting one out of 4500 women. It is characterized by utero-vaginal aplasia with normal development of secondary sexual characteristics and normal 46, XX karyotype.
Case Report
A 65-year unmarried female has presented with high grade endometrioid carcinoma of left ovary with capsular invasion.
Discussion
Ovary is usually present with variable degree of dysgenesis, and ovulation occurs normally. Ovarian cancers have rarely been described in this syndrome. We describe here a rare case of MRKH syndrome with ovarian cancer to aware surgeons about this possibility. Knowledge of this complication will help treating surgeons to keep these patients on regular follow-up.