We report the case of a 59-year-old woman admitted for a sudden headache due to a subarachnoid haemorrhage. On CT scan, the clots predominated into the posterior fossa without high-density in the sylvian or interhemispheric fissures. The vertebral angiography revealed a dural arteriovenous fistula at the foramen magnum associated to an aneurysm of the cervical anterior spinal artery. Due to the high rebleeding risk of a dural shunt, we proposed curative treatment using microsurgical interruption of the intradural draining vein. On the postoperative angiography at 15-day follow-up, the 2 malformations were corrected and the outcome at 6 months was excellent. Based on the literature, we assess this exceptional association and suggest its possible management.