Lymphomatoid granulomatosis is a vasocentric lymphoreticular proliferative disease. Some patients go on to develop frank neoplasia. Central nervous system involvement with lymphomatoid granulomatosis has been reported previously in the literature. We are presenting a childhood case evaluated with magnetic resonance imaging and x-ray computerized tomography who developed severe tissue destruction with pleomorphic cellular infiltrate and oligoclonal light chains. This report adds information regarding neuroimaging as well as immunopathology data relevant to the basic biology of this disease.