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Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) or (Müllerian Agenesis) represents uterovaginal aplasia or hypoplasia of unknown etiology in young women with usual 46, XX karyotype, and normal secondary sexual characteristics. We report a 15-year-old adolescent woman with primary amenorrhea, and normal pubertal secondary sexual characteristics, normal hormonal workup, and clinical examination. Abdominopelvic magnetic resonance imaging (MRI) revealed cervical and uterine agenesis, with the absence of the proximal thirds of the vagina. Both kidneys are fused in the right iliac fossa with oval lobulated appearance, picture of crossed fused ectopia. The ovaries are normal and located bilaterally. The diagnosis of MRKHS type 2 was confirmed based on clinical, biochemical, and radiological findings. The correct clinical and radiological diagnosis of MRKHS by MRI is crucial for the next steps in long-term management.
Keywords: Müllerian Agenesis, Uterovaginal malformations.
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