Cardiovascular disease is common in patients with obesity hypoventilation syndrome (OHS) and accounts in part for their poor prognosis. This narrative review article examines the epidemiology of cardiovascular disease in obesity hypoventilation syndrome, explores possible contributing factors and the effects of therapy. All studies that included cardiovascular outcomes and biomarkers were included. Overall, there is a higher burden of cardiovascular disease and cardiovascular risk factors among patients with obesity hypoventilation syndrome. In addition to obesity and sleep-disordered breathing, there are several other pathophysiological mechanisms that contribute to higher cardiovascular morbidity and mortality in OHS. There is evidence emerging that positive airway pressure therapy and weight loss have beneficial effects on the cardiovascular system in obesity hypoventilation syndrome patients, but further research is needed to clarify whether this translates to clinically important outcomes.