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Few previous studies have been conducted on the severe traumatic brain injury (sTBI)-associated coagulopathy in children. The purpose of this study was to evaluate the incidence and risk factors of sTBI coagulopathy in a pediatric cohort and to evaluate its impact on outcomes.Retrospective analysis of pediatric patients (younger than 18 years) sustaining isolated sTBI [head Abbreviated Injury Scale (AIS) score ≥3 and extracranial injuries AIS score <3]. Criteria for sTBI-associated coagulopathy included thrombocytopenia (platelet count <100,000 per mm3) and/or elevated international normalized ratio >1.2 and/or prolonged activated partial thromboplastin time >36 seconds. Incidence and risk factors of sTBI coagulopathy and its impact on in-hospital outcomes were analyzed.Overall, 42.8% (n = 137) of the 320 patients studied developed coagulopathy, with increasing incidence in a stepwise fashion with escalating head AIS score (31.1, 46.2, and 88.6% for head AIS score 3, 4, and 5, respectively; p < 0.001). Depressed GCS, increasing age, an ISS ≥16, and brain contusions/lacerations were independently associated with the presence of coagulopathy. The case fatality rate was 7.8% (n = 25); 17.5% versus 0.5% in coagulopathic versus noncoagulopathic patients, respectively. After logistic regression to adjust for confounders, no statistical significant mortality difference in patients with and without coagulopathy was noted (adjusted p = 0.912).Incidence of coagulopathy in children suffering isolated sTBI is exceedingly high at 40% and reflect the head injury severity. A low GCS, increasing age, ISS ≥16 and intraparenchymal lesions proved to be independently associated with TBI coagulopathy.