Rituximab is a chimeric murine-human monoclonal antibody that targets the CD20 antigen found on B cells and results in rapid depletion of this cell population. It is indicated for patients with relapsed or refractory, low-grade or follicular, CD20-positive, B cell non-Hodgkin's lymphoma. In addition, rituximab has been used for many other diseases, including refractory pemphigus. In this study, 42 case reports of patients with refractory pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus treated with rituximab were reviewed for clinical efficacy and safety. Forty-one of the 42 patients had at least some improvement following the rituximab therapy, while 6 suffered infectious adverse events. Though rituximab appears to be effective in the treatment of refractory pemphigus diseases, further studies are warranted to clarify its overall safety, especially concerning the risk of infectious adverse events in this patient population.