A nine-year old myxedematous girl with short stature, isosexual pseudoprecocious puberty and bilateral multicystic ovaries underwent pelvic laparotomy due to an acute abdomen because of adnexal torsion. She also delayed bone, age, severe hypothyroidism, hyperprolactinemia, hyperestrogenism and pituitary adenoma. All of these findings led to the diagnosis of Van Wyk Grumback Syndrome, which is a rare condition characterized by long standing untreated hypothyoidism, pseudoprecocious puberty and nulticystic ovaries. Adminsitration of levothyroxine efected gradual regression and reversal of symptoms. This case highlight the significance of recognizing the clinical features of this syndrome in order to medically treat the child and thus, prevent its undesirable effects in the physial andmental growth an dpossible surgical cimplications that ay affect her future reproductive capacity.