Lymphomatoid granulomatosis is rare EBV mediated lymphoproliferative disorder occurring within the 4th to 6th decade, with most risk factors relating to an immunocompromised state. Histological analysis shows an angiocentric and angiodestructive infiltrate with varying degrees of necrosis and atypical EBV positive cells. The lungs are the primary site of involvement in over 90% of cases but central nervous system involvement can occur alongside or in isolation. Neurological presentation is varied and MRI commonly displays focal white matter parenchymal lesions. A brain biopsy may be required to exclude competing diagnoses or in the absence of identifiable disease elsewhere. Treatment depends on stage of disease and can range from observation, withdrawal of immunosuppressive regimes or aggressive chemo/immunotherapy