Hepatopulmonary syndrome (HSP) is characterized by the triad of advanced liver disease, arterial hypoxemia, and intrapulmonary vascular dilatation. Most cases of HSP are associated with cirrhotic portal hypertension; however, it has also been reported in acute liver failure patients. An estimated prevalence of HPS in patients with chronic liver disease is around 5-32%. Sarcoidosis is a granulomatous disorder with noncaseating granulomas involving the lungs in 90% of cases. Sarcoidosis can involve the liver but in only about 1% of cases does it lead to cirrhosis and portal hypertension. In this review, we report a rare case of liver cirrhosis due to sarcoidosis with associated hepatopulmonary syndrome and discuss this in detail.