Haemophilia A is an inherited bleeding disorder, commonly involve soft tissues and joints. Gastrointestinal tract
bleeding, are not uncommon but seldom highlighted. A 23-year-old male with underlying severe haemophilia A was
presented with a generalised abdominal pain for 2 days, abdominal distension, diarrhoea and vomiting. He did not
have any trauma to the abdomen. Abdominal examination revealed generalized tenderness with sign of guarding
on palpation. Laboratory investigations revealed isolated, prolonged activated partial thromboplastin time (APTT)
with normal total white blood cell count and haemoglobin level. In view of acute abdomen, which was not resolved
by conservative treatment, an emergency laparotomy was done with FVIII concentrate and recombinant factor VII
(rFVII) coverage. Intraoperative findings noted patchy gangrenous spots of about 30 cm in length in the small bowel.
Histopathology examination revealed an evidence of haemorrhage within the submucosal and intramuscularis layer
from the resected specimen. This case highlighted the possibility of gastrointestinal bleeding without prior trauma,
which can be presented as acute abdomen in severe haemophilia patient.