OBJECTIVE Although ocular manifestations are common among patients with Behçet's disease, corneal perforation has not been reported in the literature. We report an unusual case of Behçet's disease with corneal perforation as the main ocular involvement. RESULTS An elderly Chinese patient was referred for eye examination because of clinical suspicion of Behçet's disease. Eye examination showed evidence of long-standing autoimmune disease of the eye, mild iritis and a corneal perforation in the left eye. Despite evidence of chronicity and corneal perforation, the patient was asymptomatic. The perforation was successfully treated with cyanoacrylate glue. CONCLUSIONS While anterior segment involvement is common in Behçet's disease, this case highlights an unusual and hitherto unreported ocular involvement.