We recently performed mitral valve repair for a case of mitral regurgitation due to Barlow's disease, which is relatively rare in Japan. Both the anterior and posterior leaflets were affected by advanced myxomatous change, and appeared markedly thickened and redundant. Although extensive resection of the anterior leaflet is not a generally accepted method, nearly one fourth of the anterior leaflet was resected in this case. Now, at 36 months after the operation, there is only trivial regurgitation and the patient is doing well and without symptoms. We believe that extensive resection of the anterior leaflet can be a useful treatment for repair of a redundant anterior leaflet with excess tissue.