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Introduction: The Mayer-Rokitansky-Küster-Hauser syndrome is characterized by the absence of the vagina and uterus and primary amenorrhea.Objective: The diagnostic evaluation and the therapeutic possibilities of a rare syndrome.Materials and methods: We present a case of a Rokitansky syndrome.Results: The patient presented normal development of secondary sexual characters. Analysis also showed normal hormonal function behavior along with a 46, XX karyotype. Magnetic resonance imaging revealed uterine agenesis and treatment was delayed until the patient and her family understood the diagnosis.Conclusions: The diagnostic suspicion is the milestone to manage this disease. Physical exploration, hormonal function, and radiological studies must confirm this syndrome. Laparoscopy is the approach of choice. 
Muñoz, M. del M., Noguero, R., & Martín, S. (2011). Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Diagnostic and therapeutic approach of a rare disease. Colombia Medica, 42(3), 369–372.


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Received 2011-09-26
Accepted 2011-09-26