The so-called Dyke-Davidoff-Masson syndrome (DDMS) is a rare disorder of 
cerebral hemiatrophy. The clinical presentation may consist of facial asymmetry, 
contralateral atrophy (including the trunk, and the extremities) and 
hemiparesis, speech difficulties, mental retardation, and epilepsy. Because it 
involves multiple systems, especially problem of the airway, occult myopathy, 
and seizure disorder, anesthesia for such a patient is a challenge to any 
anesthesiologist. However, there are no clinical reports which concern the 
anesthetic management of such patients in the literature. We herein report a 
5-year-old girl, a sufferer of DDMS, scheduled for burr trephination. The 
successful anesthetic management is brought forward with highlights of inference 
from the experience.