Aim: Behçet's disease is a multi-systemic autoimmune disease, characterized with recurrent oral-genital ulcers and uveitis. Neuro-Behçet's disease (NBD) may rarely cause tumour like brain lesions. Methods:Here we present a case of forty-two-year-old Turkish male with tumour like brain lesion whose tissue biopsy result was compatible with necrotic inflammation, perivascular polymorphonuclear leukocytes (PMNL), lymphocytes and other inflammatory cells infiltration. Bacterial and fungal blood cultures were negative. The patient was diagnosed with NBD and was treated with intravenous methylprednisolone 1000 mg/day for 5 days. His neurologic examination improved significantly with the treatment. We evaluated histopathological properties of all biopsy proven cases reported so far in the literature including our case.Results: When tumour-like lesions were seen in patients who had Behçet's disease in past medical history, NBD should be considered for preventing unnecessary brain operation. Seventy-three percent of all biopsy proven tumefactive NBD cases had inflammatory cell infiltration in the tumour like lesion area. Conclusion:After excluding infectious aetiology, high dose IV methylprednisolone should be begun as soon as possible in spite of mass effect that is compatible with tumour in brain magnetic resonance imaging (MRI); the good response to steroid supports to the diagnosis of tumefactive NBD. Although most tumefactive NBD patients have inflammatory infiltration in biopsy material why some cases do not have any inflammatory cell infiltration in acute lesion area needs to be clarified in further research.