A ten-week-old male Golden retriever puppy was presented with severe hyperesthesia, mild neurological deficits and episcleral bleeding. Clotting times showed a normal prothrombin time (PT) and prolonged activated partial thromboplastin time (aPPT). Computed tomography (CT) of the vertebral column showed intradural, extraparenchymal hyperattenuating changes on precontrast CT images and epidural mass lesions, suggestive of hematorrhachis. Hemophilia A was confirmed by a low-factor VIII activity (FVIII:C). Although the dog improved clinically with intravenous analgesia and cage rest, it was euthanized by the owners' choice because of the risk of developing future episodes of spontaneous hemorrhage. In young male puppies with severe hyperesthesia and mild neurological deficits, hemophilia A should be considered as a possible differential diagnosis.