Abstract:This is the first reported case of CCHS in China. The infant, of gestation age 36 weeks, had dyspnea immediately after birth. Through the course of the disease, the infant manifested hypercapnia and hypoxemia induced by hypoventilation. These manifestations were improved by mechanical ventilation. However, abnormal blood gas findings were noted after weaning of ventilatory support or by altering ventilator parameters. On day 6 of his life, the infant's autonomous breathing druing wakefulness improved, with an increase in respiratory rate to 36 times per minute, although hypoventilation still existed. However, during sleep, there was a decrease in the respiratory rate to 15 times per minute, as well as arrhythmia and sternal retractions. The infant survived for 14 days on ventilation support. These symptoms are consistent with previously overseas reported cases of CCHS.