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Patients with factor XIII (FXIII) deficiency present with a bleeding tendency that is difficult to diagnose because their coagulation test results are normal. We herein report a case of a 74-year-old male who presented to our hospital in cardiac arrest. After resuscitation, he was found to have sigmoid volvulus and necrosis; therefore, an emergency laparotomy was performed. Intraoperative findings revealed an extensive strangulated ileus in addition to sigmoid volvulus. We performed resection without reconstruction and maintained open abdominal management (OAM) for six days. After abdominal closure, the patient experienced postoperative bleeding four times from the mesenteric transection; three of the bleeding episodes required open hemostasis. Since he had mild coagulopathy during each bleeding episode, FXIII deficiency was suspected and diagnosed. After administration of FXIII concentrate, the tendency to intraoperative bleeding improved significantly. FXIII deficiency should be considered in cases of repeated severe bleeding, even when coagulation tests reveal no major abnormalities., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Omoto et al.)