Background: In the literature we found only five reports about noninvasive ventilation in cases with central hypoventilation syndrome.
Patient and method: We report about a 4-year-old boy with severe late onset hypoventilation syndrome. During an interval of 3 months with nasal mask ventilation during sleep he showed an excellent cognitive and statomotoric development. After this time, he needed a noninvasive ventilation with a negative pressure system.
Results and discussion: In our opinion, noninvasive nasal mask ventilation is a modern method in the treatment of patients with central hypoventilation syndrome. Tracheotomy is only necessary during the first year of life.