Hepatomegaly is a common manifestation in Gaucher disease. In some patients with the disease, hepatic fibrosis and portal hypertension are observed. A patient with Gaucher disease with the hepatopulmonary syndrome associated with severe cyanosis and hypoxemia was examined for intrapulmonary right-to-left shunt using Tc-99m MAA. Quantitative evaluation revealed an approximately 50% right to left shunt as indicated by Tc-99m MAA activity in the lungs and systemic organs.