Background Vascular involvement is an infrequent clinical manifestation of Behçet’s syndrome. Owing to the rarity of arterial involvement in Behçet’s syndrome, there is limited experience in managing this phenomenon. Case presentation Here, we report a 28-year-old Iranian man with a Behçet’s syndrome background, who presented with shoulder pain and hoarseness. Chest computed tomography angiography was conducted with a suspicion of a vascular pathology causing pressure on the recurrent laryngeal nerves. The patient was diagnosed with a ruptured innominate artery pseudoaneurysm. An innominate artery to the right common carotid artery bypass was performed, and the pseudoaneurysm was excised and replaced with an expandable polytetrafluoroethylene graft. Eventually, the patient was discharged after an uneventful hospital course. Conclusion It appears that we are still a long way from finding the optimal treatment for Behçet’s syndrome vascular involvement, and a combination of surgical and medicinal treatments is required.