Acute chest syndrome in sickle cell disease is a form of acute lung injury that may progress to acute respiratory distress syndrome and death. Despite recent advances in diagnosis and treatment that have resulted in improved survival in sickle cell disease, acute chest syndrome remains the most common cause of death in this population. The current standards of treatment for acute chest syndrome have been reviewed. Biomedical re-search forms the basis for sound clinical decision making and implementation of interventions that target prevention, diagnosis, and effective treatment options. Although current clinical trials are ongoing to address several new potential therapeutic options,more research using preventative and interventional strategies in sickle acute lung injury is warranted.