Epilepsy is a common childhood neurologic disorder. In 2007, epilepsy affected an estimated 450,000 children aged 0-17 years in the United States. Approximately 53% of children with epilepsy and special health care needs have co-occurring conditions, and only about one third have access to comprehensive care. The few studies of mortality risk among children with epilepsy as compared with the general population generally find a higher risk for death among children with epilepsy with co-occurring conditions but a similar risk for death among children with epilepsy with no co-occurring conditions. However, samples from these mortality studies are often small, limiting comparisons, and are not representative. This highlights the need for expanded mortality surveillance among children with epilepsy to better understand their excess mortality. This report describes mortality among children with epilepsy in South Carolina during 2000-2011 by demographic characteristics and underlying causes of death. The overall mortality rate among children with epilepsy was 8.8 deaths per 1,000 person-years, and the annual risk for death was 0.84%. Developmental conditions, cardiovascular disorders, and injuries were the most common causes of death among children with epilepsy. Team-based care coordination across medical and nonmedical systems can improve outcomes and reduce health care costs for children with special health care needs, but they require more study among children with epilepsy. Ensuring appropriate and timely health care and social services for children with epilepsy, especially those with complications, might reduce the risk for premature death. Health care providers, social service providers, advocacy groups and others can work together to assess whether coordinated care can improve outcomes for children with epilepsy.