Barlow's disease is a complicated form of degenerative mitral valve (MV) disease. Infective endocarditis (IE) often occurs on the basis of primary heart diseases and may be combined with valve perforations. Cleft‐like indentations (CLIs) were suggested by Ring et al. in 2013. They are located at the inter‐scallop position and involve at least one‐half of the valve. Herein, we report a case of Barlow's disease combined with IE and CLIs, which was confirmed intra‐operatively and by histopathological examination. The CLIs were misdiagnosed by two‐dimensional transthoracic echocardiography as perforations, but rightly interpreted by preoperative three‐dimensional echocardiography. The possibility of CLIs should be considered in the evaluation of mitral regurgitation caused by myxomatous MV diseases.