Dr. Kumar S**, Dr. Lakshmaiah V*,Dr.Karthik Naidu.K.C.*** **Asst.Prof,Dept. of Medicine,Sri Devaraj Urs Medical College and Research Center,Tamaka,Kolar,Karnataka,INDIA. *Prof. & Medical Superintendent,Dept. of Medicine,Sri Devaraj Urs Medical College and Research Center,Tamaka,Kolar,Karnataka,INDIA. ***Junior Resident,Dept. of Medicine,Sri Devaraj Urs Medical College and Research Center,Tamaka,Kolar,Karnataka,INDIA. Corresponding Author:Dr.Kumar.S , Asst.Prof.,Dept. of Medicine,Sri Devaraj Urs Medical College,Tamaka, Kolar, Karnataka, INDIA-563101 Mob:9900981096 ,email: kumarsrinivasan66@gmail.com Abstract: Dyke – Davidoff – Masson Syndrome (DDMS), also called as cerebral hemiatrophy, is a rare clinical condition characterized by seizures, facial asymmetry, contralateral spastic hemiplegia or hemiparesis, with learning difficulties. It is commonly diagnosed in presence of associated radiologic findings which include cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Here we report a case of DDMS in a 15 year old female patient who presented with seizures and mental retardation. Computerized Tomography of the brain showed hemi atrophy involving the left cerebral hemisphere. An X-ray of the Para nasal air sinuses was normal.