A 76-year-old man was admitted to our hospital because of dyspnea. Three years before admission, dyspnea was recognized and had been given a diagnosis of interstitial pneumonia by a general physician. A year later, he received home oxygen therapy. After admission, we found that he had alcoholic liver cirrhosis and an increased alveolar-arterial oxygen level in his arterial blood gas. Moreover, he had an intrapulmonary vascular shunt, detected by contrast-enhanced echocardiography and perfusion scan with ^99mTc-macroaggregated albumin. These results confirmed hepatopulmonary syndrome. Furthermore, exhaled nitric oxide (NO) was elevated in the patient although he had never had bronchial asthma or any other allergic diseases. Animal models of hepatopulmonary syndrome have shown that exhaled NO is associated with dilated vessels. To the best of our knowledge, this paper describes the first case of hepatopulmonary syndrome with elevated exhaled NO in Japan.