Congenital central hypoventilation syndrome (CCHS) is a rare, idiopathic disorder characterized by a failure of automatic respiration. Abnormalities such as seizure disorder, failure to thrive, and Hirschsprung disease have been associated with CCHS. In this report, the authors discuss the use of vagal nerve stimulation (VNS) to treat a medically refractory seizure disorder in a child who had previously undergone placement of bilateral phrenic nerve stimulators for treatment of CCHS. Concomitant use of phrenic and vagal nerve stimulators has not previously been reported in the literature. No adverse reactions were noted with both devices working. Diaphragmatic pacing (DP) was clinically unaffected by VNS. The patient experienced a marked reduction in seizure frequency and severity following vagal nerve stimulator placement. Based on this case, the authors conclude that VNS is a potentially safe and efficacious treatment option for seizure disorder associated with CCHS in patients undergoing DP.