Background. The purpose of this study was to define the early and midterm results obtained after the use of edge-to-edge repair with mitral annuloplasty in the setting of Barlow's disease. Methods. Between 1998 and 2004, 41 patients having Barlow's disease had an edge-to-edge repair creating a double-lumen mitral valve orifice in our unit. In 38 patients (93%), an annuloplasty band was also inserted. Results. Preoperatively, all patients had severe mitral regurgitation (MR), 12 were in New York Heart Association (NYHA) class I, 15 in class II, and 14 in class III. One patient died in hospital (2.4%) and 5 experienced complications (12.5%). There were no late deaths. At follow-up, 2 patients had severe MR and underwent valve replacement, 1 exhibited moderate MR, and 5 had mild MR. Kaplan-Meier 5-year survival, freedom from reoperation and recurrent moderate-severe MR was 97.6% ± 2.4%, 94% ± 4.4%, and 90.6% ± 5.1%, respectively. At latest echocardiographic evaluation (mean 35 ± 12 months) the mean left ventricular end-systolic and end-diastolic diameters, and the mitral valve area decreased (p = 0.0001) compared with baseline. The mean mitral valve gradient increased (p = 0.001) without clinical evidence of mitral stenosis whereas ejection fraction did not change. Currently, 35 patients are in NYHA class I and 5 are in class II. Conclusions. In the setting of Barlow's disease, use of edge-to-edge repair with mitral annuloplasty is safe and provides lasting restoration of mitral valve competence with measurable hemodynamic and clinical benefits. In our unit, it is the procedure of choice for correction of MR in patients having Barlow's disease. © 2005 by The Society of Thoracic Surgeons.