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Case report: GJML, 2 months, male, born 39 weeks, without complications in the perinatal period. When he was 1 month old, the first seizure was observed, with gaze deviation up and clonic movements of the limbs. He had clonic seizures and tonic seizures, daily. Twenty days later, the patient began infantile spams in flexion. Physical examination revelead macrocefalia, ipsilateral hemifacial hypertrophy with a mass in lower half of left cheeck (lipoma), and skin lesion in cervical region, cervical hipotonia and pyramidal signs. The neuroimaging showed gross asymmetry with enlargement of one hemisphere, dysplastic cortex, and asymmetry and deformity of the ventricular system. The EEG showed asymmetric suppressionburst pattern. Conclusion: The authors report a rare case of a hemimegalencephaly with some peculiarities. An early asymmetric suppression-burst may be observed in newborns and young infants with the clinical picture of Ohtahara syndrome in only 5 % of the patients. In the clinical case described, also there was observed a overlap syndrome between Ohtahara-West.

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