Mayer-Rokítansky-Küster-Hauser (MRKH) syndrome: Diagnostic and therapeutic approach of a rare disease

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.