Introduction: An association of polymicrogyria, drop attacks and electrical status-epilepticus during sleep is rare. The objective of the present study was to investigate this association in tertiary center setting and elucidate its profile.  Methods: Retrospective record review of 5 years from Pediatric neurodevelopment unit and epilepsy clinic was done. Clinical details, neuroimaging, serial electroencephalogram recordings and outcome were recorded for children with this entity. Results: Two children fulfilled the diagnosis. Both boys had global developmental delay, hemiplegic cerebral palsy and focal seizures. Age of onset of focal epilepsy was 1.5 year and 2.5 year. Neuroimaging showed right sided polymicrogyria in both the cases. The onset of drop-attacks was at the age of 4 and 3.5 year respectively. Corresponding electroencephalogram showed electrical status-epilepticus during sleep. Both had associated cognitive decline and behavioral abnormalities with the onset of electrical status-epilepticus during sleep. The condition remained refractory to multiple anti-epileptic drugs and immune-modulatory therapy. Conclusions: The association of electrical status-epilepticus during sleep and polymicrogyria is rare in childhood and refractory to anti-epileptics.