Abstract: Dyke–Davidoff–Masson syndrome (DDMS) is a rare epilepsy syndrome which is characterized by cerebral hemiatrophy with ipsilateral compensatory skull changes and contralateral hemiplegia recurrent. Here we reported a case of which the curative effect turned out to be unsatisfied after conservative treatment, physical examination revealed mental retardation, facial asymmetry, mild right hemiparesis, MRI scan of the head showed left cerebral hemiatrophy, calvaria thickening, PET-CT showed less functional left cerebral hemisphere. The patient finally underwent multi-lobe disconnection to relieve recurrent seizure, whose seizure was satisfactorily controlled in the following up of one year.

Key words: Dyke–Davidoff–Masson syndrome, cerebral hemiatrophy, seizure, multi-lobe disconnection