 Cardiac saccharosis is a rare but potentially life-threatening condition that can occur in patients with biopsyproven extracardiac saccharosis. Although electrocardiography and trans thoracic echocardiography are often used to rule out cardiac involvement, they may not detect all cases of cardiac saccharosis. Therefore, physicians should be aware of this population and consider further testing if necessary. This article was authored by Hiroshi Ohaira, Tokahiro Sotto, Osomu Manabe, and others.