Treatment of autoimmune bullous diseases can often be challenging and primarily consists of conventional systemic corticosteroids and other immunosuppressant agents. Rituximab is a chimeric monoclonal antibody against human CD20 leading to a transitory B-cell depletion with a lowering of autoantibody levels. Applications for autoimmune diseases have emerged in recent years and case reports support the use of rituximab in different autoimmune blistering disorders. Of the patients treated, 90% showed significant clinical improvement. In about a third of these patients, a clinical remission requiring further immunosuppressive medication was achieved, and in about a quarter, complete long-term remission was induced. About one third of patients suffered from serious adverse events after rituximab treatment. The combination of rituximab and intravenous immune globulin or immunoadsorption appears to be advantageous in severe cases. Randomized controlled trials are necessary to better determine the efficacy and adverse effects of rituximab in the treatment of autoimmune blistering diseases.