<prompt> A 19-year-old patient complains of primary amenorrhea. She had well developed breast and pubic hair but on examination there was absence of uterus and vagina. Likely diagnosis is :XYYMullerian agenesisGonadal dysgenesisKlinefelter syndrome  <response>: Developmental defect of the Mullerian ducts resulting in the condition described as the Mayer-Rokitansky-Kuster-Hauser MRHK syndrome. The MRHK syndrome occurs in 1:5000-1:20,000 women at bih, and is diagnosed in approximately 1:1500 gynaecologic admissions. The clinical features include primary amenorrhoea, paial or complete absence of vagina, a wide array of uterine abnormalities, skeletalrenal and other associated abnormalities, a normal female appearance and secondary sexual characteristics and a normal 46 XX karyotype. The ovaries are anatomically and functionally normal. These patients seek medical consultation because of primary amenorrhoea or in case of presence of functional uterus 1:10 cases--because of cyclic abdominal pain occurring as a result of occult menstruation. Pelvic ultrasonographyMRI and laparoscopy help to establish the diagnosis. Mullerian duct agenesis may result in the uterus represented by a nodule rudimentary uterus with hypoplastic or dimple vagina. Reference: Shaws Textbook of gynaecology,16th edition page no 132 