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Congenital central hypoventilation syndrome (CCHS) is a rare condition. The main characteristic is respiratory insufficiency during sleep. Patients who have CCHS need varying degrees of ventilation support during sleep, or even all day long, according to its severity. We report a term baby with repeated extubation failure, CCHS and Hirschsprung’s disease diagnosed at 1 month of age. This patient was discharged at 5 months old with a home ventilator and reached normal developmental milestones.