OBJECTIVE The performance of the Ross procedure in the case of geometric mismatch between pulmonary autograft and a bicuspid aortic root has not yet been fully evaluated. To prevent geometrically caused autograft dysfunction, a modification of the surgical technique is necessary. METHODS Between January 1996 and January 2007, 50 patients (33 male, 17 female; mean age 50+/-14 years; range 13-63 years) underwent replacement of a diseased bicuspid aortic valve (stenosis in 14 cases; insufficiency in 21; combined disease in 15) with a Ross procedure. The pulmonary autograft was inserted partially in supra-annular position to correct the geometric mismatch between the deeper base of the non-coronary sinus and the right/left coronary sinus. In 24 of these patients, additional tailoring of the non-coronary sinus was necessary. In eight patients the non-coronary sinus was covered with a glutaraldehyde treated autologous pericardial patch to prevent pseudoaneurysm formation. Patients were followed up 1, 2, 5 and 10 years postoperatively. RESULTS There were no early or late deaths. There were six reoperations. One patient was reoperated because of persistent severe aortic valve insufficiency 9 months postoperatively. Three patients were reoperated for formation of subannular pseudoaneurysm, 6, 9 and 30 months postoperatively. One patient was reoperated for closure of a paravalvular dehiscence. Another patient was reoperated 1 year postoperatively because of a severe pulmonary stenosis due to excessive calcification of the bioprosthesis. Echocardiographic follow-up of the remaining patients showed no evidence of residual or recurrent pulmonary autograft regurgitation or progression of aortic root dilatation. CONCLUSIONS Autograft replacement of the bicuspid aortic valve is challenging, as the geometric mismatch has to be adjusted. Valve dysfunction is avoided by a supra-annular implantation technique, but pseudoaneurysm formation at the base of the non-coronary sinus is a worrying aspect. Patch reinforcement may solve this issue.