Pemphigus vulgaris (PV) affecting the pediatric population is extremely rare, representing less than 3% of all PV cases. Conventional treatment with systemic steroids with or without adjuvant immunosuppressants used in adult cases of PV is often employed. However, pediatric cases often present a therapeutic challenge given their increased susceptibility to systemic therapy’s associated risks. To date, no specific guidelines regarding treatment strategies in this patient population exist. We describe a 14-year-old Hispanic female patient with severe, recalcitrant PV with suboptimal treatment response to systemic steroids and intravenous immunoglobulin (IVIG) successfully treated with rituximab (RTX), showing control of disease in as early as 2 weeks after treatment initiation. To our knowledge, only 45 cases of pediatric PV successfully treated with RTX have been reported in the literature, highlighting its use as a safe therapeutic option in this patient population
