Congenital adrenal hyperplasia (CAH) may be an etiologic factor for premature pubarche in about 12% of the cases, with the 21- ydroxylase deficiency as the most likely cause. A 4 ½ year old girl presented with premature pubarche and shortly thereafter developed severe chronic vulvovaginitis. She was suspicious as having a form of CAH since her brother also had premature pubarche. Though her androgens were elevated (i.e., testosterone and dehydroepiandrosterone sulfate as was her brother’s) both of them had low 17- ydroxyprogesterone levels ruling out the non-classic 21 hydroxylase deficiency and neither were hypertensive ruling out the 11-beta hydroxylase deficiency. A serum 17-hydroxypregnenalone level could not be obtained on the female sibling because she was taking prednisone for asthma, but one could be obtained on the male. Indeed the 17-hydroxypregnenalone level was increased suggesting that they had the very rare form of non-classic CAH, the type II 3-hydroxysteroid dehydrogenase deficiency. The question now was how to treat her rare severe vulvovaginitis. Since her mother had manifestation of the increased cellular permeability syndrome with pelvic pain, headaches, and chronic fatigue and was successfully treated with dextroamphetamine sulfate, the female sibling was also treated with dextroamphetamine sulfate. Not only did her vulvovaginitis quickly disappear, but she showed marked improvement in her attention deficit disorder (ADD) and edema causing weight gain. Similarly, her brother showed significant improvement in his fatigue, weight gain and ADD. Her mother did not have premature pubarche, and thus the results suggest that though one may think that two rare conditions, i.e., premature pubarche and pediatric vulvovaginitis, occurring in the same patient may have a common etiology, the authors conclude that they are probably independent conditions.