Tuberous sclerosis is an autosomal dominantly transmitted genetic disorder that has characteristic symptoms triad of mental retardation, convulsion, and facial angiofibroma. Because it is a relatively uncommon disorder, there are few informations about anesthetic management for this disorder. We report a case of tuberous sclerosis that had involved bilateral kidneys. A 14-year old female patient was induced with thiopental sodium, rocuronium bromide and then intubated with an internal diameter 6.5 mm of reinforced tube. Anesthesia was maintained with O2-N2O-isoflurane. Surgery took 3 hours and there were no remarkable changes in vital signs and arterial blood gas analysis.