Objective To investigate the clinical and pathological features of lymphomatoid granulomatosis(LG).Methods The data of clinic and the results of pathological study of brain biopsy which stained by immunohistochemical method were reviewed in one case with LG.Results The patient was presented with headache,dizzy,slurred speech and myasthenia of limbs.MRI showed abnormal signals on bilateral cerebral white matter,pons,cerebellum and cervical-thoracic spinal cord.Gd-enhanced imaging showed punctiform enhancement.The protein and IgG in cerebrospinal fluid elevated slightly.Brain biopsy showed angiocentric,angiodestructive granuloma formation combined with infiltration of multiple cells.The infiltrated cells confirmed immunohistochemically were lymphocytes and mononuclear phagocytes.In situ hybridization technique showed EBV positive.The status of this patient improved after radiotherapy and glucocorticosteroid treatment.Conclusion LG lacks characteristic features in clinic,laboratory and radiological examinations,so the pathological examination is important for diagnosis of this disease.