Acquired haemophilia is a rare disorder caused by the development of autoantibody to factor VIII. It is sometimes associated with malignancies, and usually appears during disease course. In rare instances, acquired haemophilia is the presenting manifestation of a malignant disease. We report a 76-year-old man, who presented with spontaneous haematomas of his four limbs. A factor VIII inhibitor was found and the patient diagnosed with acquired haemophilia. Initial etiologic diagnostic workup including a thoracic and abdominal computed tomographic scan was negative. Factor VIII inhibitor disappeared on corticosteroids and factor VIII level normalized. Seven months later, the patient died from a multimetastatic cancer. About 15% of acquired haemophilia are associated with malignant disease (malignant lymphoma or solid neoplasia). Although rare, the development of a factor VIII inhibitor few months before the diagnosis of the malignant disease raised the issue of the appropriate initial investigations and further monitoring to recommend these patients. We propose a regular clinical monitoring and a thoracic and abdominal computed tomographic scan at six-month follow-up to screen for malignant disease.