Tuberous sclerosis is a neurocutaneous syndrome with a wide variety of clinical, pathologic, and radiologic manifestations. Intracranial phakomatosis has been reported to include subependymal nodules; cortical tubers, and subependymal giant-cell astrocytomas. Subependymal giant-cell astrocytomas are rare, benign brain tumors of unknown histogenesis which cause increased intracranial pressure, seizures, and focal neurologic signs. The only treatment of these tumors is total surgical removal, because they are not sensitive to irradiation and chemotherapy. We report a patient with tuberous sclerosis who underwent surgery for a large subependymal giant-cell astrocytoma.