A case of diencephalic syndrome in a girl is reported. Computed tomography revealed a midline low-density mass accompanied by ventricular dilatation. Preoperative hormonal examination showed a high level of serum growth hormone, a low level of serum prolaction and lack of diurnal variation in serum cortisol levels. Several partial removals of the tumor and radiotherapy enabled the patient to survive for 12 years after onset. Histological examination revealed fibrillary astrocyma, and the DNA histogram showed a diploid pattern. Combination of multiple surginal removal and radiotheraphy thus appears useful for prolongation of survival in case of diencephalic tumor.