Rare Association of Mayer–Rokitansky–Kuster–Hauser Syndrome with Rectovestibular Fistula and Colonic Atresia

A BSTRACT Rectovestibular fistula is the most common presentation of female anorectal malformation. Its association with Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome and Colonic atresia is uncommon. We present the staged management of a case of a female neonate with MRKH syndrome with rectovestibular fistula and colonic atresia. This is probably the first such case ever to be documented in literature. An absent vaginal opening with rectovestibular fistula should raise suspicion of MRKH syndrome. Detailed clinical and radiological evaluation with meticulous planning of the staged procedure is necessary for a successful outcome.