Sickle cell anemia is a lifelong debilitating disease often required multiple, frequent hospital admissions. Multiple organ systems become damaged, but the heart, although demonstrating abnormalities, is relatively spared. With increasing life span of these patients, cardiac dysfunction may become more prominent. Recent noninvasive and pathologic studies have given new information on the effect of this disease on the heart. This article reviews the current knowledge of cardiac involvement in sickle cell anemia.