Obesity hypoventilation syndrome is the most frequent cause of chronic hypoventilation and is increasingly more common with rising obesity rates. It leads to considerable morbidity and mortality, particularly when not recognized and treated adequately. Long-term nocturnal noninvasive ventilation is the mainstay of treatment but evidence suggests that CPAP may be effective in stable patients. Specific perioperative management is required to reduce complications. Some unique syndromes associated with obesity and hypoventilation include rapid-onset obesity with hypoventilation, hypothalamic, autonomic dysregulation (ROHHAD), and Prader-Willi syndrome. Congenital central hypoventilation syndrome (early or late-onset) is a genetic disorder resulting in hypoventilation. Several acquired causes of chronic central hypoventilation also exist. A high level of clinical suspicion is required to appropriately diagnose and manage affected patients.