Between 1977 and 1991, at the Children’s Hospital of Philadelphia, 10 patients, 5–16 years of age, were diagnosed as having subependymal giant cell astrocytomas. These patients accounted for 1.4% of all pediatric brain tumors seen during that time interval. One patient received a course of radiation therapy, which was ineffective in preventing tumor growth. All underwent surgical resections with the goal being maximal tumor debulking, if not complete resection. In 6 patients this was accomplished by the frontal transventricular route and, in the more recent patients, surgery was performed using a transcallosal approach. There were 2 perioperative deaths, and 2 other patients died later of causes unrelated to tumor progression. The remaining 6 patients remain alive and stable at a mean of 6.7 years of follow-up (range 1.8–12.4). None of these patients has received additional radiation therapy. Two patients have no other evidence of tuberous sclerosis. The use of modern radiographic and surgical techniques has made the treatment of this disease safer than in the past.