Objective To explore the clinical manifestations and differential diagnosis of pituitary hyperplasia caused by primary hypothyroidism in children.Methods A patient with pseudo-pituitary macroadenoma caused by hypothyroidism who received treatment in Hetian District People's Hospital,Xinjiang Uygur Autonomous Region,China was reported.Meanwhile,relevant literature was searched in Pubmed to sort out the clinical and imaging manifestations of pituitary hyperplasia caused by primary hypothyroidism and the differences from pituitary tumors.Results This patient almost had to be surgically treated.After thyroid hormone replacement therapy,the growth and development of the patient recovered,and the pituitary gland was reduced.Conclusion For patients with enlarged thyroid and pituitary glands,a comprehensive assessment and thyroid hormone replacement therapy are required.After follow-up for 3 months,re-evaluation is performed to determine whether there are indications for surgery to avoid permanent hypophyseal hypofunction caused by surgical resection.