Hemiconvulsion–Hemiplegia–Epilepsy (HHE) syndrome is an uncommon outcome of prolonged focal status epilepticus in childhood usually occurring during febrile illness followed by hemiplegia ipsilateral to the side of convulsions which is associated with seizures, cerebral holo–hemispheric global atrophy of one hemisphere and transient or permanent epilepsy. This is a report of 4 year male child with right sided hemiparesis, vacant stare and adrenal insufficiency with past history of altered sensorium and focal dyscognitive seizures. This is accompanied by radiologic evidence of acute cytotoxic edema in the affected hemisphere followed by chronic atrophy. MRI brain shows gliosis, restricted diffusion in the left cerebral hemisphere and with reduced caliber of he left ICA, anterior, middle and posterior cerebral arteries.