A case of craniopharyngioma confined in the third ventricle is reported. A 53-year old man was admitted because of headache, nausea, vomiting and general malaise lasting for one year and a half before admission. Neurological examinations showed no significant abnormalities except for a slight degree of papilledema. Lumbar cerebrospinal fluid contained 200 mg/dl of protein and other laboratory tests were normal. Plain skull films were normal. A static technetium 99m brain scan showed no increased uptake. Angiography demonstrated only symmetrical dilatation of the lateral ventricle. Conray ventriculography outlined an irregular mass almost completely filling the anterior third ventricle. Pneumoencephalography showed no abnormality in the suprasellar cistern. Biplane CT scans showed a homogeneously highly enhanced tumor located precisely above the sella turcica and in the third ventricle. A right frontal craniotomy was performed and the transcallosal approach to the lateral ventricle was made. A part of the tumor protruded through the left dilated foramen of Monro into the lateral ventricle. The tumor was reddish, solid, soft and densely adherent to the right wall of the third ventricle. The tumor was subtotally removed under an operating microscope. Histopathological examination of this tumor revealed squamous cell type craniopharyngioma. The patient made an uneventful postoperative recovery, but 2 months after the first operation. a V-P shunt was performed because of gradually developing normal pressure hydrocephalus. Although he recovered after the shunt operation, he died of pneumonia 2 months after the shunt operation. No autopsy was performed. Craniopharyngioma located entirely in the third ventricle has, to our knowledge, been reported in only six cases previously. These reports were reviewed and the condition is discussed in terms of etiology, diagnosis, clinical course and treatment.