Eighteen cases of biopsy-proven pediatric diencephalic gliomas are reviewed in terms of clinical presentation, radiographic features and pathological findings. Treatment included radiation therapy (RT) in 14, chemotherapy in 4 and conservative management in 3. Actuarial survival and quality of life are analyzed. We conclude that: (1) thalamic involvment portends a poor prognosis both in terms of histology and survival, (2) beneficial effects of RT are difficult to demonstrate and (3) therapy for pediatric diencephalic gliomas should be individualized and long-term spontaneous remissions may occur.