Five children with tuberous sclerosis and intracranial tumors were surgically treated at Neurosurgery Department of Hacettepe University School of Medicine. Two of the five patients presented with the giant cell astrocytoma filling the third ventricle. Direct surgical intervention and tumor removal was carried out in all four patients (three with a total surgical resection and one with a subtotal resection) and cerebrospinal fluid shunting procedure in one. Histopathological examination revealed giant cell astrocytomas in four patients. Postoperatively, one died and the rest four patients survived with minor focal neurologic difficulties. The surgical results and the changing concepts in the treatment of tuberous sclerosis are discussed and the literature is reviewed.