Von Willebrand disease (VWD) is a hereditary bleeding disorder characterized by a deficiency of von Willebrand factor (VWF). In pediatrics, plasma-derived VWF (pdVWF) is the standard for factor replacement when indicated. Alternatives are needed for kids who are refractory or intolerant to pdVWF. There is limited data on the safety and efficacy of recombinant VWF in the pediatric population, and none regarding the switch from pdVWF to rVWF due to intolerance. Herein, we describe the safe and effective use of rVWF in a pediatric patient with history of anaphylaxis to pdVWF, who underwent elective orthopedic surgery.