Diaphragmatic pacemaker is a device that reduces or eliminates the need of mechanical ventilation in patients with chronic respiratory failure who keep the phrenic nerve-diaphragm axis intact, as long as they do not present intrinsic lung disease. Although its implantation has been practiced for decades, its use is not widespread and to date, there is little published literature about it, mostly related to high spinal cord injury and congenital central hypoventilation syndrome. Objective: To describe an experience of diaphragmatic pacemaker implantation in a pediatric patient with acquired central hypoventilation syndrome. Clinical Case: Female patient with central hypoventilation syndrome secondary to ischemic brainstem lesion as a result of ventriculoperitoneal shunt malfunction. For this reason, for 5 years she was supported by inpatient mechanical ventilation. At 7 years of age, a diaphragmatic pacemaker was implanted by thoracoscopic surgery, which allowed, after a period of rehabilitation and respiratory conditioning, mechanical ventilation withdrawal, and hospital discharge. Conclusions: Diaphragmatic pacemaker is a feasible, potentially safe, and cost-effective option for decreasing or eliminating mechanical ventilation dependence and improve life quality in patients with acquired central hypoventilation syndrome.