Portopulmonary hypertension (PoPH) is a form of pulmonary arterial hypertension in the setting of cirrhotic or noncirrhotic portal hypertension. It is a significant complication affecting approximately 5% of patients with cirrhosis. PoPH contributes to morbidity and mortality of chronic liver disease by significantly increasing right cardiac afterload, ultimately leading to right heart failure and complicating disease management. We review the current literature of PoPH pathophysiology with special focus on cardiopulmonary hemodynamics, clinical presentation, diagnostic challenges, and management strategies. A central illustration should visually capture the summarized information.