Haemophilia A and B are X-linked bleedingdisorders included among the rare diseases and causedby mutations in the factor VIII (FVIII) and factor IX(FIX) genes. Acute compartment syndrome (ACS) of the limb refersto a constellation of symptoms, which occur following a risein the pressure inside a limb muscle compartment. Here we are reporting a rare case of Haemophilia A patient presenting with Acute compartment syndrome and the sequential steps in the management which include emergency fasciotomy, debridement and flap cover.
Conclusion: Haemophilia presenting as ACS is rare and a high index of suspicion is needed to diagnose it. The results are favourable when the diagnosis is made early.