Schilder’s disease is a rare sporadic demyelinating disease of the brain. We report a 6year old boy with Schilder’s disease who presented with progressive right hemiparesis. Brain magnetic resonance imaging (MRI) showed a single large tumefactive white matter lesion. Based on clinical features and pre and post treatment MRI findings, a diagnosis of Schilder’s disease was made. The patient showed dramatic clinical improvement and significant regression of the lesion in response to high–dose pulse steroid.