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Subependymal giant cell astrocytoma(SEGA) is a rare intraventricular tumor that mainly occurs in patients with tuberous sclerosis. The tumor characteristrically arises in the wall of the lateral ventricle and presents as an intraventricular mass causing hydrocephalus by obstruction of the foramen of Morno. Hemorrhagic presentation of SEGA has been very rarely reported only in patients with tuberous sclerosis. A 41-year-old man presented with sudden onset of severe headache, nausea and vomiting. There was no clinical feature of tuberous sclerosis. Preoperative neuroimaging studies showed a intraventricular tumor with hemorrhage. The mass was totally removed throught interhemispheric transcallosal approach. The authors report an unusual case of hemorrhagic SEGA, which is devoid of any clinical feature of tuberous sclerosis with review of pertinent liteatures. Key words:Subependymal giant cell astrocytoma;Hemorrhage;Intraventricular mass;Tuberous sclerosis.