A case of craniopharyngioma with unusual location confined within the third ventricle is reported. The 56 years old adult presented with symptoms and signs of increased intracranial pressure. There were no characteristic findings of craniopharyngioma in X-ray examinations, but computed tomography scan showed a mass lesion in the third ventricle. Literature survey revealed nine cases of craniopharyngioma developed solely within the third ventricle.