Sickle cell lung disease presents a challenging care paradigm involving acute and chronic lower airway disease, sleep-disordered breathing, pulmonary vascular disease, and modification by environmental factors. Understanding the presentation, pathophysiology, and diagnostic approaches is essential for accurate identification and management. While significant progress has been made, there remains a need for research to develop effective treatments and interventions to decrease disease burden in these children. Additionally, the long-term impact of interventions on cardiopulmonary outcomes is unknown. Collaborative efforts among health care providers, researchers, advocacy groups, and policy makers are crucial to improving the lives of children with SCD.