Objective To improve the diagnosis level of MRI for hypothalamic hamartomas by analyzing its clinical and MRI features. Methods MRI and clinical data of 8 patients (included 5 boys and 3 girls, age from 1 to 14 years old.) with hypothalamic hamartomas proved by operation and pathology were reviewed retrospectively. All patients were examined with pre and post contrast MR imaging. Results The clinic symptoms included precocious puberty (n=5), gelastic seizure (n=3), other types of seizures (n=4), polydipsia and polyuria (n=1). MRI showed all masses were located in the suprasellar and interpeduncularis cistern regions with spheroidal or irregular shape .All masses were closely related to the tuber cinereum or the mammillary body, diameter ranging from 12mm to 25mm.The masses presented as iso-isointense to gray matter on T1-weighted imaging and T2-weighted imaging, or slightly high intense on T2- weighted imaging, without enhancement by gadolinium. Conclusion The clinical and MRI features of hypothalamic hamartomas are characteristic, which is helpful for diagnosis.