Cerebral mass-like lesion (MLL) is a rare form of Neuro-Behcet's (NB) disease. There is currently no detailed knowledge on this issue in the literature. Our aim was to describe a Behcet's disease (BD) patient with MLL, followed by a clinical analysis in light of the available literature regarding BD patients who suffered from an MLL or tumefactive lesion in the brain. We conducted a review of the English literature to analyse data on MLL in BD. The Pub-Med, Web of Science, Proquest and Ovid databases were searched for articles or abstracts using the term "Behcet's disease" combined with one of the following terms: mass-like lesion, tumour-like lesion and tumefactive lesion. We compared clinical and laboratory features of BD patients with MLL with NB patients. We found 12 cases plus our case (6 male, 7 female; mean age: 40 years) with BD who developed MLL alongside BD. Five out of 13 BD patients (38%) had a history of BD before the onset of neurological symptoms. In 8 patients (62%), BD was diagnosed after the onset of neurological involvement. Headache, hemiparesis, dizziness, aphasia, nausea and vomiting were the presenting manifestations of NB patients with MLL. Genital ulceration, eye involvement, skin lesion and arthritis/arthralgia were less commonly reported in NB patients with MLL compared to NB patients without MLL. NB disease should be considered in the differential diagnosis of cerebral MLL even when other cardinal manifestations of BD are absent. Mucocutaneous manifestations, eye and joint involvement may be seen less often in these patients.