Pemphigus is a severe blistering disorder caused by autoantibodies to desmogleins 1 and 3. Some patients with pemphigus never enter into remission with conventional therapy of high-dose corticosteroid. These patients need steroid-sparing agents such as conventional immunosuppressants or immunobiologics. Rituximab is an anti CD20, chimeric monoclonal antibody binding to the CD20 antigen on B cells, which proved to be effective in recalcitrant pemphigus. A 54-year-old Korean woman presented with recurrent episodes of multiple erythematous bullae and macules with ulcerations on trunk, upper extremities and oral mucosa and her disease did not respond so effectively to systemic corticosteroid or cyclosporin. The patients were treated with four weekly infusions of 375 mg/m2 of rituximab but lesions did not improve for a while. After low dose steroid maintenance therapy was added to the rituximab treatment, the skin lesions improved rapidly and subsided without any recurrence. Now she is administered mycofenolate mofetil 500mg /d. Herein, we report a case of recalcitrant pemphigus vulagris, which was successfully treated with Retuximab and low dose steroid maintenance therapy.