ABSTRACT
Mayer-von Rokitansky-Kuster-Hauser syndrome [MRKH], comprises of combined hypoplasia of the vagina and the uterus, in addition it may be associated with congenital anomalies of the urinary tract and the skeleton. Its main clinical presentation is primary amenorrhoea in the presence of normal secondary sexual characteristics. Multi nodular goiter [MNG] is one of the most prevalent thyroid disorders worldwide and sometimes impairs health and well being. We report a 33-year-old Saudi female with features of the atypical form of Mayer-von Rokitansky-Kuster-Hauser syndrome [MRKH] syndrome, large multinodular goiter with compression of trachea causing obstructive symptoms, hirsutism and hyperprolactinaemia. Although the concurrence of MRKHS and MNG appears to be coincidental, this association has not been previously reported and the association with endocrine abnormalities such as hyperprolactenaemia or hirsutism is rarely described