In this paper the authors describe a case of central hyperventilation syndrome associated with a neural crest tumor. The girl had several apneic spells at the age of three years. She had a tracheostomy and mechanically assisted ventilation while asleep. Routine hematologic studies and chest x-ray were within normal limits. Computerized tomography scan and magnetic resonance imaging revealed a left paraspinal mass suggestive of a neural crest tumor. This mass was excised and histopathologic findings of this tumor revealed a ganglioneuroblastoma.