The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital hypoplasia or aplasia of the uterus and agenesis of the upper part of the vagina. Here, we report the case of severe abdominal pain that was diagnosed as torsion of the right rudimentary uterine horn with multiple leiomyomas in a patient with MRKH syndrome. The twisted rudimentary uterine horn and multiple leiomyomas were successfully removed via laparoscopy. MRKH syndrome with uterine horn torsion is uncommon and ours is the first case of torsion in MRKH syndrome treated via a laparoscopic approach. Our report indicates that laparoscopy is ideal for the simultaneous diagnosis and treatment of pelvic massin MRKH syndrome.