Background: Behçet's disease is a chronic inflammatory disease that involves systemic organs and vascular structures. Arterial system involvement is rarer than the venous system, and it is one of the major causes of death. Vertebral artery (VA) involvement is rare and there are a limited number of reported cases in the literature. This report aimed to present the imaging findings of a case of Behçet's disease with a giant aneurysm originating from the right VA. Case Report: A 35-year-old woman with a 10-year history of Behçet's disease applied to our center with complaints of pain on the right side of the neck. CT angiography showed a 7.5x6.5 cm sized sac-cular aneurysm in the superior of the right hemithorax adjacent to the mediastinum, with contrast filling the V1 segment of the VA. In our case, the VA aneurysm had no relation to the subclavian artery and showed filling from the contralateral VA. The described findings were demonstrated in Doppler examination, phase-contrast MRI, and conventional and CT angiography. Conclusion: Behçet's disease is a chronic inflammatory disease that can involve the arterial system. Although VA involvement has not been reported frequently, this vasculitic process may affect all arterial structures.