OBJECTIVE Double-orifice tricuspid valve (DOTV) is an extremely rare congenital anomaly. By analysing the feature of its diagnosis and surgical treatment, we want to summarise the clinical experience of treating DOTV. METHODS Review two cases of DOTV treated by us between August 2009 and December 2011. One case was diagnosed as partial atrioventricular septum defect, and the other was tetralogy of Fallot. The defects were both identified during the operation for other congenital cardiac malformations and both accessory orifices were normal. But one of them was sutured because of its possible effect in future. RESULTS Cardiac colour Doppler echocardiogram was made at three to five days after operation and all results were normal. No operative complication or late deaths occurred. The time of follow-up were one month, three months, six months, one year and two years after operation, and all examinations were normal. CONCLUSIONS The accessory orifice of DOTV patients has its own independent chordae tendinea and mastoid muscle. So the gap of tricuspid valve should be excluded and the classification should be amended according to it. It should be surgically treated, when there is of dysfunction with it or potential harmful effect in sequent treatment.