Conditions causing central alveolar hypoventilation are uncommon in children, but have a profound impact on those affected. Congenital Central Hypoventilation Syndrome is defined by a mutation in PHOX2B, resulting in a variable amount of hypoventilation, primarily during sleep. Rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation usually presents later in childhood with rapid weight gain and hypoventilation. Other conditions, including familial dysautonomia, Chiari malformations, and Prader-Willi syndrome and acquired conditions such as brain tumors, trauma, and encephalitis, can also result in central hypoventilation. Treatment options include mechanical ventilation and diaphragmatic pacing, which can improve survival and quality of life.