Ocular mucous membrane pemphigoid presents some of the most challenging dilemmas in anterior segment management. Diagnosis is made difficult by the insensitivity of immunopathological investigations and the differential diagnosis of other scarring conjunctival disorders. The management of the associated ocular surface disease involves control of blepharitis, dry eye, filamentary keratitis, keratinization, lid malposition, and persistent epithelial defect, as well as the identification and avoidance of toxicity. Inflammation associated with the underlying disorder demands the use of systemic immunosuppressive therapy in many patients. New biological immunotherapies have been used when conventional immunosuppressive therapies fail. Ophthalmic plastic surgery is essential for the management of lid malposition and corneal exposure. Improving vision may require the use of specialized contact lenses, cataract surgery, and corneal and ocular surface reconstructive surgery. All surgery must be integrated with ocular surface treatment and immunosuppressive treatment to avoid disease exacerbations.