Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by failure of embryologic growth of the mullerian ducts resulting to agenesis or hypoplasia of the uterus and upper part of the vagina while both ovaries and fallopian tubes are normal. Various associated malformation with MRKH syndrome are noted in literature, with a renal system anomaly as the most common. However, adnexal tumors in MRKH syndome are rare. To date there have been no reported cases of fallopian tube tumors in patients diagnosed with MRKH. This paper presents a case of an 18 year old nulligravida who presented with primary amenorrhea secondary to MRKH sydnrome, with an associated Papiliary Serous Cystadenofibroma of the right fallopian tube Management of the case as well as review of related literature are presented. 

Cystadenofibroma