We reported a 39-year-old man with Dyke-Davidoff-Masson syndrome presenting with 
total hemiatrophy. The patient had a muscle defect in the left occipital lesion 
at birth and left hemiatrophy including the face in his infancy. The present 
illness consists of atrophy of the left face, trunk and extremities, a muscle 
defect accompanied with scleroderma of the left dorsal cervical lesion, 
bilateral pes cavus, cleft palate, left hemiparesis, including facial palsy and 
diminished superficial sensation of the left side of the body. Decrease in right 
cranial volume and slight elevation of the right temporal pyramid and the 
superior border of the orbit were seen on the X-ray study of the head. Atrophy 
of the right cerebrum and cerebral peduncle was seen on magnetic resonance 
imaging. These findings suggest that hemiatrophy of the brain was responsible 
for total hemiatrophy of the body in this patient. The etiology of cerebral 
hemiatrophy is not known. There is only a report of Dyke-Davidoff-Masson 
syndrome accompanied with total hemiatrophy.