Purpose To describe clinical characteristics of Behçet's disease in Tunisia. Methods It's a retrospective and multicentric study conducted by the Tunisian society of internal medicine. Inclusion criteria were those of the international study group. Were also included patients without international study group criteria but with at least one manifestation among arthritis, venous thrombosis or neurological manifestation with oral and genital ulceration or oral ulceration and skin lesions. Results Five hundred and nineteen patients were included. 87.5% of them fulfilled the international criteria. The male to female ratio was 2,7. The mean age was 28.7 ± 9.3 years at onset and 32.7 ± 9.2 years at diagnosis. The incidence of each manifestations was as follows: oral ulcers: 100%, genital ulcers: 87.5%, pseudofolliculitis: 67.6%, erythema nodosum: 17.5%, positive pathergy test: 51%, joint involvement: 55%, uveitis: 32.2%, vein thrombosis: 24.9%, arterial aneurysms: 3.9%, neurological involvement: 11.6%. The frequency of HLA B51 antigen was 35% among the 187 patients tested. There was no difference in the manifestations of the disease between patients having B51 and those lacking it. Venous thrombosis (29.8 vs 11.4%), arterial involvement (4.4 vs 1.4%) and uveitis (37.5 vs 17,9%) were significantly more frequent in men whereas erythema nodosum (22.9% vs 15.6%) and joint involvement (70,7 vs 49.9%) more frequent in women. The mean follow up was 6,1 ± 5.7 years. Mortality rate was 2.3% in our series. Conclusion Our study confirms the androtropism of the disease in Mediterranean and Middle east countries. Positive pathergy test and venous thrombosis were more frequent in our study, like those from Mediterranean region. Whereas, ocular and neurological involvement were quite less frequent in our series.