Objectives Enthesitis has been reported in Beh & ccedil;et's disease (BD); however, the analysis remains insufficient. This study examined the clinical characteristics of patients with BD presenting with enthesitis.Methods A single-centre, observational, retrospective cohort study was conducted by reviewing medical records. Cases of BD with enthesitis were registered, and BD cases without enthesitis served as a control group. Statistical analysis included the Mann-Whitney U test, Fisher's exact tests, and logistic regression analysis.Results A total of 31 cases of BD with concomitant enthesitis were identified. The Achilles tendon was the most commonly affected site (n = 26, 83.9%), and arthritis frequently co-occurred (93.5% vs 66.7%, P = .002). Multivariate analysis comparing BD cases without enthesitis (n = 93) revealed associations of enthesitis with arthritis, pustular skin lesions, and epididymitis (P = .031, P = .036, and P = .043, respectively). The treatment response for enthesitis in BD was favourable.Conclusions Enthesitis in BD was associated with arthritis, pustular skin lesions, and epididymitis, aligning with the previously reported cluster involving pustular skin lesions, arthritis, and enthesitis. Management of BD should incorporate enthesitis considerations, and further research is essential to refine diagnostic and treatment strategies.