Patients with chronic bullous disease of childhood (CBDC) generally respond well to treatment with drugs like Dapsone and oral and topical steroids. Refractory cases are additionally treated with immunosuppressants like azathioprine, methotrexate, ciclosporin, colchicine, tetracycline, and IVIG (Intravenous Immunoglobulin). However, there are few patients who fail to respond to these therapies and pose a challenge both to the patient and the treating physician. Rituximab has been used widely to treat pemphigus cases in adults, but its use in children has not been much reported. We hereby report a case of a 5-year-old child with CBDC, refractory to other treatments, who responded favorably to Rituximab.