BackgroundAtlantoaxial dislocation (AAD) is the most common craniovertebral junction malformation (CVJm) which are anomalies of the bones and soft tissues surrounding the foramen magnum. It usually leads to neurologic abnormalities because of instability of this mobile area. But vertebral artery dissection (VAD) caused by AAD is uncommon.Case reportWe report a 15-year-old boy who presented with acute onset of bilateral VAD leading to posterior circulation ischemic stroke (PCIS). Computed tomography angiography (CTA) indicated dissection and occlusion of bilateral intracranial vertebral arteries and AAD with os odontoideum. After antithrombotic treatment for 3 months, the patient got complete revascularization and received posterior C1-C2 fusion.DiscussionThere have only been tens of cases of PCIS caused by CVJm. We reviewed these relevant literatures and suggested that more attention should be paid to vascular impairment for patients with CVJm.