To investigate the clinical features and potential risk factors of coronary involvement in Behçet's disease (BD). In this case-control study, we retrospectively reviewed medical records of BD patients admitted to our institute from 2000 to 2016. Coronary involvement was documented by coronary angiography and (or) computed tomography angiography. We analyzed the demographic, clinical, and laboratory data and compared with age- and gender-matched BD patients without coronary involvement. Among 476 BD patients (296 males) enrolled, 19 (4.0%) patients (17 males) were diagnosed with coronary involvement. The median duration from onset of BD to coronary involvement was 2.8years. Coronary stenosis, aneurysm, and occlusion were presented in 13, 9, and 3 patients, respectively. Multiple coronary artery stenoses and aneurysms were observed in 9 and 3 patients, respectively. Smoking (36.8%) was the major traditional risk factors. Male gender (89.5% vs 61.1%, p = 0.01), skin lesions (78.9% vs 55.3%, p = 0.08), pathergy reactions (36.8% vs 10.5%, p = 0.01), extra-cardiac vasculitis (36.8% vs 6.6% p< 0.01), elevated ESR (57.9% vs 34.2%, p = 0.01), and elevated CRP (63.2% vs 42.1%, p < 0.01) were more common in BD patients with coronary involvement comparing with those without coronary involvement. Multivariate analysis confirmed pathergy reaction (OR = 3.81, 95% CI 1.08-13.47) was the independent risk factor. Coronary involvement in BD patients is rare and male-predominant and is characterized by the aneurysm and multivessel involvement. Elevated ESR and CRP are frequent, and the pathergy reaction is the independent risk factor. Key Points • Coronary involvement in BD patients is rare and male-predominant. • Pathergy reaction is the risk factor for coronary involvement in BD patients.