Mucous membrane pemphigoid (MMP) is a pemphigoid disease defined by the presence of autoantibodies against the dermal-epidermal junction and predominant involvement of mucous membranes. Diagnosis is made by the clinical presentation and linear deposits of IgG and/or IgA and/or C3 at the dermal-epidermal junction by direct immunofluorescence microscopy of a perilesional biopsy. Circulating autoantibodies can be detected in most patients by indirect immunofluorescence microscopy on salt-split human skin as well as ELISA and immunoblotting with recombinant and cell-derived target antigens. For systemic treatment of MMP, corticosteroids, dapsone, mycophenolates, and azathioprine are applied. In severe cases and in cases with rapid disease progression cyclophosphamide, rituximab, high-dose intravenous immunoglobulins, and immunoadsorption are used. For the successful management of MMP patients, close cooperation with dentists, ophthalmologists, ENT specialists, gynecologists, and gastroenterologists is essential.