Uterine and vaginal agenesis or the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital who can be classified into type I (isolated) or Rokitansky syndrome, and type II (associated with malformations of organs of the renal, skeletal, cardiovascular, and other systems). The most common clinical presentation for MRKH syndrome is primary amenorrhea. Women with MRKH syndrome typically have normal ovarian function and a 46,XX karyotype.We report the case of a young girl who consults for primary amenorrhea and has utero-vaginal aplasia associated with a spinal defect with a single ectopic right kidney.

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