Oral and genital ulcers, and cutaneous vasculitic lesions are considered hallmarks of the disease, and often precede other manifestations. Mucocutaneous lesions figure prominently in the presentation and diagnosis. Therefore, their recognition may permit earlier diagnosis and treatment, with beneficial results for prognosis. This review overviews the current state of knowledge regarding the therapeutic approaches including local and systemic agents for the treatment of mucocutaneous lesions of BD.