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Background: Tranexamic acid (TXA) is a medication used to treat or prevent excessive blood loss due to certain medical conditions. It has a low side effect profile and is safe to administer in most instances. Anaphylaxis cases due to intravenous TXA have been reported in the literature. We report the first pediatric case of anaphylaxis due to the use of nebulized TXA. Case Presentation: A 2-year-old boy with cerebral palsy, epilepsy, and tracheostomy was hospitalized with pneumonia. On the fourth day of hospitalization, the patient started bleeding from the trachea. Nebulized TXA was started to reduce tracheal bleeding. Anaphylaxis developed 5 min after administration of nebulized TXA. Subsequently, the patient was successfully treated with adrenaline, intravenous fluids, antihistamines, and steroids. Conclusion: Nebulized TXA is increasingly used off-label. Although it has a safe profile, side effects such as anaphylaxis may occur rarely. It is essential to recognize the symptoms of anaphylaxis when using nebulized TXA.