Background: Ankylosing spondylitis was reported to associate with an increased risk of cerebrovascular diseases. In this article, we aimed to report the first case of ankylosing spondylitis associated with moyamoya disease treated with encephalo-duroarterio-synangiosis. Case presentation: A 9-year-old boy with ankylosing spondylitis appeared a symptom of repeated transient ischemic attacks which performed as left hemiparesis. Magnetic resonance angiography showed a typical finding of left anterior cerebral artery, bilateral middle cerebral arteries and the supraclinoid portion of the right intal carotid artery stenosis with an abnormal vascular network at the base of the brain, diagnosed with moyamoya disease. He received a medication treatment but did not underwent revascularization surgery. After three months, ankylosing spondylitis symptoms got some relief, whereas transient ischemic attacks was more frequency. Due to the bad cerebral blood flow on acetazolamide computed tomography perfusion and poor clinical manifestation, he underwent a right encephalo-duroarterio-synangiosis. Postoperatively, the symptoms of transient ischemic attacks disappeared. Conclusions: We reported the first case of ankylosing spondylitis associated with moyamoya disease. Moyamoya disease could appear in patients with ankylosing spondylitis. Revascularization surgery probably is an effective treatment for preventing preoperative ischemic events recurrence.