Mucous membrane pemphigoid (MMP) is an autoimmune blistering, scarring and occasionally mutilating disease that may progress to blindness or airway obstruction. Over the past few years, rituximab (RTX) has emerged as a potential therapeutic solution for MMP; however, the literature regarding its efficacy in the treatment of severe MMP is sparse. We studied four patients with recalcitrant MMP who were treated with RTX. Three of these had recalcitrant laryngeal disease; two were unresponsive to RTX, while the third patient achieved complete remission (CR) but relapsed twice. The fourth patient, who had oral and ocular disease, also achieved CR. In addition, we reviewed 143 cases of MMP treated with RTX reported in the literature to date. Of these, 120 had late observation endpoints, of whom 81 (67.5%) achieved CR, 24 (20%) received partial remission and 15 (12.5%) had no remission. Based on this study, the presence of laryngeal MMP seems to predict refractoriness to RTX treatment. In conclusion, we found that RTX can ameliorate the MMP course and that laryngeal involvement, which is known to be a prognostic factor for severe MMP, may also predict poor response to RTX.