Résumé : In 1937, the Turkish dermatologist Hulusi Behçet described the vasculitis syndrome that bears his name : a triad of aphtous stomatitis, genital ulceration and uveitis. Thromboses are also a serious complication of BD and may occur early. The ocular and CNS manifestations can be extremely incapacitating. The cause is unknown. Genetic factors have long been implicated in the disease. Behçet's disease is strongly associated to the presence of the HLA B51. laboratory findings are diagnostic of BD with a non specific increase in acute phase reactants. The disease course is characterized by remissions and relapses and the lack of evidence-based data for its treatment. There are no controlled studies evaluating treatment of BD in children, and physicians must therefore depend on the experience gained in treating adults.