Survival for children with brain tumors less than 2 years of age at diagnosis is dismal, and the quality of life of long-term survivors poor. Between 1975 and 1987, 78 (13%) of 579 patients with brain tumors treated at Children’s Hospital of Philadelphia were under 2 years of age. Tumor site was posterior fossa in 31 (40%) and supratentorial in 47 (60%). Nine of 37 patients (24%) with malignant tumors, and 30 of 41 (73%) patients with benign tumors are alive with a mean follow-up of 116 months. Long-term survival after treatment with chemotherapy alone occurred in 10 patients, including 3 with malignant tumors. In 5 additional patients, chemotherapy delayed the need for irradiation a mean of 30 months. Of the 29 patients who relapsed after initial therapy, 12 are alive without progressive disease (2 patients with malignant tumors and 10 with benign tumors) a mean of 80 months after relapse, 2 children are alive with progressive disease, and 14 died a median of 48 months (range 9–115 months) after relapse. Twenty-one of the 39 survivors have minimal or no neurological or intellectual dysfunction. Surviving patients treated with surgery and chemotherapy have better intellectual function than patients treated with surgery and radiation (with or without chemotherapy) in that 8 of 10 children treated with surgery and chemotherapy have normal or above normal intelligence compared with 5 of 12 children receiving irradiation before their second birthday.