Patients with Sturge-Weber disease and Klippel Trenaunay syndrome may have several neurologic abnormalities. The effects of hemangiomas in the brain cortex and the spinal cord are responsible for most of the anesthetic challenges associated with this disease, particularly the risks of intracerebral bleeding, disseminated intravascular coagulation (DIC), and airway management. The successful management of this case illustrates that adequate preparation to handle the possible complications may result in safe anesthetic management in patients with these problems.