The association of gonadal dysgenesis with Mullerian tract anomalies is extremely rare and appears to be coincidental, independent of chromosomal anomalies. Gonadal dysgenesis is characterized by underdeveloped ovaries with consequent, impuberism, primary amenorrhea, and hypergonadotropic hypogonadism. Mullerian agenesis or Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in a woman with normal development of secondary sexual characteristics and a normal 46,XX karyotype. We report a case of 16 year old female patient who presented with Primary amenorrhea and Impuberism. Endocrine study revealed, hypergonadotrophic hypogonadism with normal level of TSH. The pathogenesis of the association of both is still unknown. The treatment is based on hormonal therapy, however the fertility is unfortunately compromised.