Introduction Severe status asthmaticus (SA) in children may require intubation and mechanical ventilation with a subsequent increased risk of death. In the patient with SA and refractory hypercapnoeic respiratory failure, use of extracorporeal life support (ECLS) has been anecdotally reported for carbon dioxide removal and respiratory support. We aimed to review the experience of a single paediatric centre with the use of ECLS in children with severe refractory SA, and to compare this with international experience from the Extracorporeal Life Support Organization (ELSO) registry. Methods All paediatric patients (aged from 1 to 17 years) with primary International Classification of Diseases (ICD)-9 diagnoses of SA receiving ECLS for respiratory failure from both the Children's Healthcare of Atlanta at Egleston (Children's at Egleston) database and the ELSO registry were reviewed. Results Thirteen children received ECLS for refractory SA at the Children's at Egleston from 1986 to 2007. The median age of the children was 10 years (range 1 to 16 years). Patients generally received aggressive use of medical and anaesthetic therapies for SA before cannulation with a median partial pressure of arterial carbon dioxide (PaCO2) of 130 mmHg (range 102 to 186 mmHg) and serum pH 6.89 (range 6.75 to 7.03). The median time of ECLS support was 95 hours (range 42 to 395 hours). All 13 children survived without neurological sequelae. An ELSO registry review found 64 children with SA receiving ECLS during the same time period (51 excluding the Children's at Egleston cohort). Median age, pre-ECLS PaCO2 and pH were not different in non-Children's ELSO patients. Overall survival was 60 of 64 (94%) children, including all 13 from the Children's at Egleston cohort. Survival was not significantly associated with age, pre-ECLS PaCO2, pH, cardiac arrest, mode of cannulation or time on ECLS. Significant neurological complications were noted in 3 of 64 (4%) patients; patients with neurological complications were not significantly more likely to die (P = 0.67). Conclusions Single centre and ELSO registry experience provide results of a cohort of children with refractory SA managed with ECLS support. Further study is necessary to determine if use of ECLS in this setting produces better outcomes than careful mechanical ventilation and medical therapy alone.