Management of an unusual case of atypical Mayer-Rokitansky-Kuster-Hauser syndrome, with unilateral gonadal agenesis, solitary ectopic pelvic kidney, and pelviureteric junction obstruction.

Congenital absence of uterus and vagina, Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, is mullerian agenesis and is the second most frequent cause of primary amenorrhea. Only atypical form of MRKH (type B) is associated with renal skeletal and ovarian abnormalities. We report the management of an unusual case of atypical MRKH, unilateral gonadal agenesis, and solitary ectopic pelvic kidney with pelviureteric junction obstruction (PUJO). After doing thorough Medline search, to the best of our knowledge, this is the first case reported with this combination.