Mycophenolate mofetil has been used as an immunosuppressive agent to prevent acute rejection in kidney transplantation since the early 1990s. There are several reports that mycophenolate mofetil is effective in autoimmune bullous diseases including pemphigus vulgaris in combination with high doses of systemic corticosteroids or as a monotherapy. In Japan, however, there are few reports of pemphigus vulgaris treated with mycophenolate mofetil. The present study showed that mycophenolate mofetil treatment combined with systemic corticosteroid was successful in four Japanese patients with pemphigus vulgaris who were refractory to therapies including systemic corticosteroids, plasmapheresis, and oral immunosuppressives. For Japanese and European patients, mycophenolate mofetil may be an excellent therapy in a combination with systemic corticosteroid for refractory patients with pemphigus vulgaris.