Spinal perimedullary arteriovenous fistula (AVF) or dural arteriovenous fistula (DAVF) presenting as intracranial subarachnoid haemorrhage (SAH) is uncommon. A total of 16 cases have been reported to date. A majority of the reports described cervical spinal DAVF, while two other case reports described intracranial SAH secondary to lumbar and thoracic DAVF, respectively. We report a 61-year-old Chinese man with intracranial SAH secondary to thoracic DAVF aneurysm, who presented with sudden, severe chest pain, initially suggestive of aortic dissection/acute myocardial infarction. However, a careful examination of the history and physical signs, followed by appropriate and timely investigations enabled effective treatment to be administered promptly with a good outcome. This serves to illustrate the importance of investigating the entire cerebrospinal system when neurological symptoms and clinical signs suggest extracranial primary pathology.