A valvular perforation is a well-known, and common, complication of infective endocarditis that may adversely affect the clinical outcome. However, a 'windsock' deformity of the mitral valve, as a delayed presentation of infective endocarditis, affecting the mitral valve alone, is very rare. A 42-year-old man, who underwent a mitral valvuloplasty and annuloplasty six years previously, suddenly developed pulmonary edema. He had also had a previous history of infective endocarditis, dating back three years. A transthoracic echocardiogram revealed a 'windsock' deformity of the anterior mitral leaflet (AML), resulting in an acute severe mitral regurgitation. During the operation, the AML was found to have been damaged by the previous endocarditis, resulting in an aneurysmal change of the central scallop, and a rupture of the roof. A mitral valve replacement was successfully performed, and the patient recovered uneventfully. Here, we report a rare case of a 'windsock' deformity of the mitral valve, with two perforations as a delayed complication of a healed infective endocarditis.