Ganglioglioma is a lesion rarely encountered in the suprasellar region. Here we present a case of suprasellar region ganglioglioma that was totally removed by surgery. The patient was a 15-year-old female who complained of seizure for about 1 year, polyuria and eyesight descent for about 6 months. The neurological examination was without positive findings except bi-papilledema. By MRI, a huge cystic mass lesion was found in the suprasellar region, which caused slight obstructive hydrocephalus. The size of the mass was determined to be 4 cm x 3 cm x 3 cm. The levels of GH, PRL, ACTH, TSH, LH, FSH, TT3, TT4, CORT-1 were normal. We resected this lesion totally by trans-anterior portion of the foramen of right lateral ventricle. The postoperative paraffin section and immunohistochemistry showed ganglioglioma, GFAP(+), CK(-), NF(+). The follow-up time was 10 months; the patient was in good condition and enjoyed high quality of survival. This tumor has special embryological origins, clinical manifestations, and pathological features. Ganglioglioma of the suprasellar region will be of relatively favorable prognosis if it is diagnosed and resected in the early stage.