Tranexamic acid (TXA) is widely utilized to control perioperative bleeding. TXA is considered a safe drug with few serious adverse effects, but many studies report TXA‐associated seizures, especially with cardiac surgeries. Usually, TXA‐associated seizures persist for a few minutes with no progression into status epilepticus. Here, we report, for the first time, a case of refractory status epilepticus after IV injection of TXA in a paediatric non‐cardiac surgery. This case report and literature review aim to increase awareness about TXA‐associated seizures and to provide mechanistic‐based prevention and treatment recommendations. During adenotonsillectomy for a 4‐year‐old male child, TXA infusion started after induction of anaesthesia for surgical bleeding prophylaxis. During recovery from anaesthesia, the patient developed tonic–clonic convulsions which did not improve after two IV doses of midazolam but showed an improvement after a dose of propofol. The patient did not regain consciousness and was transferred to the ICU. He had recurrent treatment‐resistant attacks of tonic–clonic convulsions. The patient developed acute kidney injury and died after 18 hours. In high‐risk patients, using the lowest effective dose with early termination of TXA infusion and prolongation of administration of anaesthetics may prevent seizures. General anaesthetics (propofol and halogenated inhaled anaesthetics) are considered the first line for prevention/treatment of TXA‐associated seizures.