Behçet's disease is a chronic, relapsing multisystemic inflammatory disorder characterized by recurrent orogenital aphthous ulcers, uveitis, and skin lesions. Vascular involvement occurs in up to 38% of these patients. Herein, we report a 19-year-old male who initially presented with an isolated celiac artery aneurysm that was treated with open surgical repair. The patient was subsequently diagnosed with Behçet's disease after the development of oral aphthous ulcers and multiple recurrent postoperative deep venous thromboses and thoracoabdominal arterial aneurysms. Ultimately, a hybrid approach was undertaken. This is the fifth celiac artery aneurysm ever reported in this patient group and the first to present with an isolated celiac artery aneurysm as the initial manifestation of Behçet's disease.