Objective: Hereditary angioedema (HAE) is a rare disorder characterized by C1 esterase inhibitor (C1-INH) deficiency or dysfunction, leading to recurrent and potentially life-threatening edema attacks. Despite advances in diagnostic tools and effective therapies, awareness among pediatricians remains limited, contributing to delayed diagnosis and suboptimal management.

Materials and Methods: A cross-sectional, questionnaire-based survey was administered to 460 pediatricians between May and August 2025. The survey consisted of five domains: demographics, educational exposure, clinical experience, knowledge assessment, and clinical practice scenarios. Data were analyzed using descriptive statistics, chi-square tests, and logistic regression to identify predictors of accurate diagnostic and therapeutic decision-making.

Results: Participants had a mean age of 38.8 ± 10.3 years, and 64.6% were female. Although 64.3% reported receiving HAE-related education, only 26.1% played a role in diagnosis and 26.3% in the treatment of HAE patients. Knowledge gaps were prominent: 51.7% recognized the absence of urticaria in acute HAE attacks, 45.0% identified bradykinin as the primary mediator, and 30.2% correctly identified autosomal dominant inheritance. For diagnostics, 44.3% selected C4 as the screening test and 67.2% selected C1-INH levels for confirmation. Only 40.7% chose icatibant as first-line therapy for acute attacks, while 43.3% incorrectly preferred antihistamines or corticosteroids. Receiving HAE-related education significantly predicted correct management responses (acute: OR 2.5; preoperative: OR 3.8; both p<0.001).

Conclusion: This survey demonstrates substantial gaps in pediatricians’ knowledge and management of HAE. Formal education and prior clinical experience significantly improved diagnostic accuracy and treatment choices. Structured training programs and guideline dissemination are urgently needed to reduce diagnostic delays and improve outcomes for children with HAE.