<p>As survival rates in sickle cell disease (SCD) continue to improve, cardiovascular complications, particularly sickle cell cardiomyopathy (SCCM), have emerged as leading causes of morbidity and mortality. SCCM represents a distinct, yet often underrecognized, cardiac phenotype characterized primarily by diastolic dysfunction, myocardial fibrosis, and pulmonary hypertension, frequently without overt systolic impairment. Its pathogenesis involves a complicated relationship among chronic anemia, hemolysis, microvascular ischemia, iron overload, and systemic inflammation. Despite its clinical importance, SCCM lacks standardized diagnostic criteria, with early detection reliant on advanced imaging techniques such as cardiac magnetic resonance imaging and speckle-tracking echocardiography, combined with biomarker profiling. Current therapeutic approaches are largely extrapolated from general heart failure management, with disease-modifying interventions like hydroxyurea and transfusion therapy offering some benefit. However, no treatment directly targets the myocardial remodeling central to SCCM progression. This review brings together what we know about SCCM, points out the challenges in diagnosis and treatment, and suggests important areas for future research, stressing the urgent need to clearly define SCCM as a separate medical condition to enhance heart health in people with SCD.</p>