Pediatric pemphigus vulgaris (PV) is a rare autoimmune bullous dermatosis that poses a diagnostic and therapeutic challenge. Rituximab, an anti-CD20 monoclonal antibody, is an important medication in adult PV but has rarely been used to treat pediatric disease. We describe successful rituximab therapy in a 4-year-old, the youngest patient with PV ever reported to receive rituximab. A live attenuated vaccination was later given without incident. We also review rituximab clinical outcomes, toxicity, dosing protocols, and relapse in children with PV.