Infantile spasms (IS) is a type of epileptic encephalopathy with poor developmental outcomes. The goal of treatment is complete control of spasms, improvement of hypsarrhythmia, and rapid reversal of delayed development. Improvements in diagnosis can result in the selection of appropriate therapy, which can improve a patient's developmental outcomes. However, there is no consensus on the best initial treatment for IS. Here, we describe 6-month-old boy with severe periventricular leukomalacia, developmental regression, and hypsarrhythmic pattern on electroencephalography, who was successfully treated with intravenous methylprednisolone.