Background: Schilder's myelinoclastic diffuse sclerosis (Schilder's disease) is a rare demyelinating disorder. Clinical features and neuroimaging findings of this disease might mimic an intra-cranial mass lesion including an abscess or a tumour.
Case report: Clinical and radiological findings of two children with the diagnosis of Shilder's disease are reported, and the role of brain biopsy as a diagnostic tool is discussed.