Surgical treatment of aortic regurgitation due to Behçet's disease is difficult. A 57-year-old male with Behçet's disease underwent aortic valve replacement with a mechanical valve for aortic regurgitation in 1995. Due to prosthetic valve detachment, 5 months thereafter he underwent a Bentall type operation with a composite graft. Due to complication of the left ventricle to pulmonary arterial fistula, 6 months later a third operation was performed for closure of the fistula. He is doing well at present 5 years after the third operation. Left ventricle to pulmonary arterial fistula is an exceedingly rare complication and has not been reported in the literature.