Ventricular tumors of tuberous sclerosis are almost always hamartomas or benign tumors occurring predominantly in children or young adults. No clear gross or histological distinctions between the ventricular “candle gutterings” and “tumors” have been identified. The clinical presentation may be acute, with the sudden onset of increased intracranial pressure and possible serious morbidity (visual impairment) or death. When these tumors are surgically removed or decompressed, the prognosis for life seems to be quite good. Malignant change in these lesions is very rare.