Purpose <p>Hemorrhagic complications after pediatric otolaryngologic surgery are well documented, yet children with bleeding disorders are frequently excluded from clinical studies. Given the prevalence and frequent incidental diagnosis of von Willebrand disease (vWD), accurate assessment of perioperative bleeding risk is essential. This study evaluated the impact of vWD on postoperative hemorrhagic complications following adenotomy (AT) and adenotonsillotomy (ATT) in children.</p> Methods <p>This retrospective cohort study included pediatric patients with confirmed vWD who underwent AT or ATT for isolated adenoid hypertrophy or adenoid hypertrophy with tonsillar hypertrophy and the control group. Demographic, clinical, laboratory, and surgical outcome data were collected. Severe postoperative hemorrhage was defined as bleeding requiring posterior nasal packing. Logistic regression analysis was performed to identify predictors of postoperative bleeding.</p> Results <p>A total of 134 children were included, comprising 42 patients in the vWD group and 92 patients in the control group. Early postoperative bleeding occurred in 11.9% of patients in the vWD group, with severe bleeding observed in 4.8%. In comparison, the incidence of severe postoperative bleeding in the general surgical population without vWD was 0.83%. The presence of vWD was associated with a nearly sixfold increased risk of severe postoperative hemorrhage (odds ratio 5.99; 95% confidence interval 1.41–25.45; <i>p = </i>0.049). No significant association was identified between baseline von Willebrand factor antigen or ristocetin cofactor activity levels and postoperative bleeding events.</p> Conclusions <p>Children with vWD undergoing AT or ATT have an approximately sixfold higher risk of severe postoperative hemorrhage compared with children without bleeding disorders.</p>