Background: Hemophilia B is a rare entity than other coagulation disorders. It is an X-linked disorder characterized by a deficiency of functionally active coagulation factor IX (FIX), resulting in spontaneous or trauma-induced bleeding primarily in joints, muscles, and soft tissues. Case Presentation: We report a case of a female who presented with a surgical problem. She had a history of massive transfusion many years back. She bled perioperatively and then we investigated her and luckily found the deficiency of FIX. She was managed and discharged home well. Conclusion: Surgeons rarely comes across this rare coagulation disorder, so this was an intriguing case in view of the unusual presentation, initial diagnostic dilemma, and challenges in management.

Keywords: Enterocutaneous fistula, hemophilia B, coagulation disorder, factor IX, case report