Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by either absence or abnormalities of the mullerian structures. It is a rare disorder, resulting in complete or partial agenesis of the uterus and cervix and primary amenorrhea. It may rarely be associated with anomalies of the urinary tract, ovaries and skeleton. Renal failure secondary to chronic tubulo-interstitial disease has been reported. We report a case of MRKH syndrome presenting late with chronic kidney disease.
Keywords: Mullerian agenesis; amenorrhea; chronic kidney disease; congenital anomalies.