OBJECTIVE This review article summarizes the recent literature in regards to advancements in the diagnosis and management of mucous membrane pemphigoid (MMP) published in the English literature since May 2011. RESULTS MMP is an uncommon, subepithelial blistering conjunctivitis that is commonly associated with vision loss. Distinct subgroups of MMP have recently been identified using advanced immunohistochemical techniques. Antiepiligrin MMP has been described as a distinct entity with a relationship to certain solid organ malignancies. There is evidence that conventional immunomodulators such as mycophenolate mofetil and biologics such as rituximab are effective in halting disease progression. Unfortunately, the majority of patients at presentation to tertiary centers already have advanced disease. Novel regenerative techniques such as stem cell therapy as well as traditional surgical approaches such as keratoprosthesis might be required for the restoration of vision in patients with significant corneal scarring. CONCLUSIONS Despite advances in available immunosuppressive treatment regimens, significant ocular morbidity persists possibly secondary to difficulty in diagnosis. Additionally, absence of clinically detectable inflammation in a significant proportion of patients causes delay in the treatment and leads to progressive scarring. Surgical management of the complications of MMP remains challenging, with high failure rates over long-term follow-up.