Abstract: 　Objective　To explore the early diagnosis and treatment of Van Wyk-Grumbach syndrome (VWGS). Methods　The clinical data of a child with VWGS were retrospectively analyzed. The related literatures were reviewed. Results　Nine-year-old female presented with growth retardation and obesity for 3 years, combined with breast development for 6 months and vaginal bleeding for 3 month. The level of free thyroxine (FT4) was 0.46 ng/dL and thyrotropin (TSH) >150 mIU/L. The GnRH stimulation test showed that the gonad axis was not activated. The serum prolactin and estradiol were significantly increased. Bone age was delayed. Color Doppler ultrasound showed enlarged uterus and enlarged ovary, and ovarian cyst was seen. Pituitary MRI showed hyperplasia of the pituitary gland. The patient received the treatment of Euthyrox, and 2 months later, thyroid function was back to normal, ovaries were significantly reduced by reexamine of color doppler ultrasound, body weight was reduced by 6 kg, and there was no vaginal bleeding. Three months later, both ovaries returned to normal size, and pituitary MRI showed hyperplasia of adenohypophysis was improved. After 6 months, both of uterus and ovary were turn to normal size, ovarian cyst disappeared, and pituitary MRI showed normal. Conclusions　VWGS is a rare complication of severe primary hypothyroidism untreated for long time and it mainly occurs in adolescent girls. Thyroid replacement therapy is effective.