Abstract Infantile bullous pemphigoid (IBP) is a rare autoimmune bullous skin disease that occurs in infants, for which standardized targeted therapeutic regimens remain unavailable. Herein, we report two infants with IBP treated with nicotinamide monotherapy. Complete clinical remission was observed within 3 weeks and 10 days after treatment initiation, respectively. Following clinical improvement, nicotinamide was gradually tapered and discontinued in approximately 2 months.