A case of a 13-years-old boy with tuberous sclerosis manifested with the complete syndrome that died with a massive intratumoral hemorrhage is presented. The post mortem examination of the brain disclosed a massive left parenchymatous brain, hemorrhage with intraventricular component. In the hemorrhage, near the lateral ventricle wall, a subependymal giant cell astrocytoma was found. This is the second case in the literature of tuberous sclerosis which cause of death was an intratumoral massive hemorrhage.