Previous reports have described the use of extracorporeal membrane oxygenation (ECMO) for acute chest syndrome of sickle cell disease (SCD). However, there have been no reports of venoarterial (VA) ECMO for cardiac dysfunction in patients with SCD. We describe a patient with SCD and life-threatening cardiogenic shock who was successfully treated with VA ECMO. Furthermore, SCD patients have unique comorbidities that warrant particular consideration when utilizing ECMO. We discuss these considerations and review the documented experience with ECMO for pediatric SCD patients from the Extracorporeal Life Support Organization (ELSO) registry. From 1990 until 2012, 52% of the 65 pediatric patients with SCD placed on ECMO survived, with 85% of those receiving venovenous (VV) ECMO surviving and 43% of those receiving VA ECMO surviving. However, significant complications, such as bleeding, neurological injury and kidney injury, also occurred with both VV and VA ECMO. Ten percent of SCD patients receiving VA ECMO experienced either a cerebral infarct or hemorrhage; our patient suffered a cerebrovascular accident while on ECMO, though she survived with good neurologic outcome. To our knowledge, this is the first report of a pediatric patient with SCD and cardiogenic shock successfully managed with VA ECMO. In conjunction with the ELSO registry review, this case report suggests that, while VA ECMO can be successfully used in patients with SCD and severe cardiovascular dysfunction, clinicians should also be aware of the potential for serious complications in this high-risk population.