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Solitary neurofibroma of the heart is extremely unusual. Few reports of neurofibroma in the left ventricle have been published. In this case report, we present the results of transthoracic echocardiography, myocardial contrast echocardiography, cardiac magnetic resonance imaging, and histopathologic examination of a patient with a neurofibroma of the heart. The patient had no evidence of any other metastasis or primary tumor in other organs, which is clinically rare.