We report two patients with severe haemophilia A, factor VIII < 2 u dL-1, diagnosed at birth, through isolated bleeding in the adrenal gland (case 1) and through a haematoma in the liver (case 2). In these two clinical cases, the vital emergency, with haemorrhagic shock, required early diagnosis of the hereditary coagulation defect. Generally at birth, any unexplained bleeding should prompt screening for haemophilia. In the newborn period, the PTT is inadequate for many reasons and the laboratory evaluation must include factor VIII and IX levels.