Primary hypothyroidism usually leads to retardation of linear growth and delay or even arrest of puberty in juvenile patients. In rare cases, pediatric patients with hypothyroidism may present with signs of VanWyk-Grumbach's syndrome (VWGS), which includes juvenile hypothyroidism, delayed bone age and pseudoprecocious puberty. The present study reported on a rare case of VWGS and other complications, including obesity, short stature, hepatomegaly and pituitary hyperplasia. In addition, a comprehensive literature review was performed to illustrate the treatment and outcome of VWGS in pediatric patients. The present study contributed to the current knowledge regarding the diagnosis and treatment of VWGS in pediatric patients.