Coexistence of pituitary macroadenoma and Mayer-Rokitansky-Küstner-Hauser syndrome.

The Mayer-Rokitansky-Küstner-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and upper part (2/3) of the vagina in women showing normal development of secondary sexual characteristics with a normal 46, XX karyotype. We report a case of a 30-year-old woman who presented with complaints of headache. Pituitary macroadenoma and MRKH syndrome were diagnosed. To the best of our knowledge, pituitary macroadenoma has not been reported in association with MRKH syndrome. However, no genetic links between MRKH syndrome and pituitary macroadenoma have been observed. Thus, the association may be incidental rather than causal.