Mayer-Rokitansky-Kuster-Hauser syndrome occurs in only approximately 1 in 4000-5000 female births. Leiomyoma is a rather common occurrence in the normal uterus, but extremely rare in mullerian remnants. Leiomyoma should be considered low on the list of differential diagnosis of pelvic mass in women with Mayer-Rokitansky-Kuster-Hauser syndrome. We have experienced a case of Mayer-Rokitansky-Kuster-Hauser syndrome associated with leiomyomas arising from both rudimentary uterine horns and report it with a brief review of literatures.