Introduction Hemimegalencephaly is a rare congenital disorder of cortical formation with hamartomatous overgrowth of all or a part of a cerebral hemisphere. This results from either increased proliferation or decreased apoptosis (or both) of developing neurons. Early infantile epileptic encephalopathy, or Ohtahara syndrome, is one of the most severe forms of age-related epileptic encephalopathies, characterized by the onset of tonic spasms within the first 3 months of life. The characteristic electroencephalogram (EEG) displays a burst suppression pattern.

Structural abnormalities of the brain are often associated. We present a case of hemimegalencephaly associated with facial and intracranial lipoma presenting as Ohtahara syndrome. Case Report A 3-month-old child of nonconsanguineous marriage, presented with a history of tonic neck flexor spasms since 20 days, in clusters, 3–4…