We present two patients with severe haemophilia A, who presented with haemorrhagic shock, secondary to a liver haematoma in the neonatal period. Both required emergency treatment and diagnosis of the hereditary coagulation defect. Major organ bleeding in severe haemophilia A in the newborn period is rare, and this unusual complication is not well recognized. Failure to recognize that the bleeding is due to a bleeding disorder, particularly in the absence of a family history, may lead to delay in appropriate management.