Abstract
Unilateral pulmonary agenesis is a rare congenital condition characterized by the absence of one or both lungs. Most of the cases have unilateral presentation as bilateral agenesis does not support life. Simultaneous Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome also has very rare presentation. It is characterized by congenital aplasia of the uterus and the vagina with normal female karyotype (46XX). A 30-year-old female, presenting with dyspnea from last 6 months and amenorrhea for last 15 years was further evaluated with gynecological examination, contrast-enhanced computed tomography thorax with the abdomen and karyotyping. She was eventually diagnosed as a case of Type I pulmonary agenesis with Type I MRKH syndrome. K
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