Background: Behçet’s disease (BD) is a rare, multisystemic inflammatory disorder with a diverse range of manifestations, primarily affecting the mucocutaneous and ocular systems. While vascular involvement is less frequent it can be severe, with coronary aneurysms being particularly rare. Case description: We report a 28-year-old male with BD who developed a giant anterior interventricular artery aneurysm. The patient, initially managed with azathioprine and colchicine, presented with acute chest pain and elevated cardiac markers. Imaging revealed a coronary aneurysm measuring 5.03 cm. Due to the inflammatory nature of the aneurysm surgical intervention was initially deferred, and the patient was treated with corticosteroids and cyclophosphamide. Despite these treatments, recurrent symptoms prompted the initiation of infliximab therapy. Administered at 5 mg/kg at weeks 0, 2 and 6, with subsequent doses every two months, infliximab led to a significant reduction in aneurysm size to 3.73 cm. Conclusion: This case highlights the effectiveness of infliximab in managing giant coronary aneurysms associated with BD, demonstrating its potential as a treatment option for severe vascular complications in BD. The substantial reduction in aneurysm size following infliximab therapy underscores the need for further investigation into its role in treating such rare but serious manifestations of BD.