Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome comprises the absence or hypoplasia of the uterus and the upper two third of the vagina. Diagnosis of these patients may be delayed until the period of adolescence or even sexualy active period because the external appearance of patients are the same with completely normal female and often do not have any complaints. Renal, cardiac, skeletal abnormalities, pulmonary and hearing defects may be associated with MRKH Sydrome. Accuracy of diagnosis of MRKH syndrome is very important for providing psychological support, acquisition of normal sexual function, treatment and to give advice on assisted reproductive techniques. In these cases, a detailed systemic evaluation for the presence of other accompanying system anomalies should not be Accuracy of diagnosis. Our presentation is about a patient with 36 year old with MRKH Sydrome diagnosed at the age of 16 and had undergone vaginoplasty operation there after she could be sexually active.
Keywords: Cyclic pelvic pain, Mayer-Rokitansky-küstner - Hauser syndrome, uterovaginal agenesia