A girl has primary amenorrhea with normal ovaries, absent internal genitalia but normal external genitalia. Most probable diagnosis?

Correct Answer: Mayer-Rokitansky-Kuster-Hausers- syndrome
Description: Mayer Rokitansky Kuster Hauser's syndrome : karyotype is 46,XX Normal secondary sexual charecteristics Functional ovaries Normal hormonal profile Associated with skeletal and renal anomalies Management involves creation of a functional vagina for coital purposes and can become biological mother by surrogacy. SHAW'S TEXTBOOK OF GYNAECOLOGY,Pg no:286,15th edition
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