Behçet's disease is a systemic vasculitis characterised by recurrent mouth and genital ulcers and uveitis. About 25% of patients suffer from vascular involvement. We describe a patient with Behçet's disease who suffered recurrent pulmonary embolism and developed severe chronic thromboembolic pulmonary hypertension. The patient was successfully treated with pulmonary endarterectomy that normalised pulmonary haemodynamics. Chronic thromboembolic pulmonary hypertension is a potential complication of Behçet's disease that may be amenable to pulmonary endarterectomy.