Tuberous sclerosis is a rare disease of autosomal dominant inheritance and may affect any organ, and less than 2% have a subependymal giant cell astrocytoma. In the present report, a 6-year-old girl with clinical features of tuberous sclerosis had an intraventricular tumor. CT scan and MRI of brain showed a tumor in the lateral ventricles extended into the Foramina of Monro and third ventricle causing severe hydrocephalus. She underwent left frontal craniotomy with partial removal of the tumor. The pathology was subependymal giant cell astrocytoma. Patient's neurological condition improved postoperatively and postoperative CT scan showed no hydrocephalus. Clinical and radiological follow-up of patients with tuberous sclerosis who had subependymal nodules in early childhood is important due to its possibility of subsequent transformation into intraventricular tumor.