International Journal of Academic Health and Medical Research (IJAHMR)   Year: 2024 | Volume: 8 | Issue: 4 | Page No.: 158-160 Le syndrome de Mayer-Rokitansky-Küster-Hauser à propos d'un cas et revue de la littérature Download PDF D. Benrahhal, Z. Hayati, Y. belhaj, S. Jayi, FZ. Fdili Alaoui, H. Chaara, A. Melhouf Abstract: : Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper two-thirds of the vagina. It is either isolated (type I) or associated with other malformations (type II or MURCS association). These anomalies can affect the kidney, the spine and, to a lesser extent, the otological sphere or the heart. The main warning sign is primary amenorrhea. Development of secondary sexual characteristics and external genitalia are normal. On the other hand, the vagina is reduced to a more or less deep cup. The ovaries are normal and functional and the hormonal status normal. The karyotype is 46.XX, with no visible chromosomal abnormality. We present a case of MRKH type I syndrome in a 36-year-old single patient who presents with primary amenorrhea with the result of radiological investigations in favor of complete agenesis of the uterus. Through this case, we study the syndrome of MRKH with a literature review.