A bilateral hilar lymphadenopathy on the chest radiograph of a 30-year-old man was pointed out in a medical examination. Four months later, he was admitted to our hospital because of exacerbation of a bilateral hilar lymphadenopathy on the chest radiograph with high fever. Physical examination revealed a marked swelling of the face and upper arms as well as a dilated jugular vein. The fever, which was his chief complaint, responded to antibiotic therapy. A CT scan of the chest confirmed the presence of bilateral mediastinal lymphadenopathy and hilar lymphadenopathy, which caused pulmonary artery and superior vena cava stenosis. A superior vena cavagram demonstrated narrowing of the vessel. Mediastinoscopy was performed for definite diagnosis. Pathologic examination of the specimen revealed noncaseating epithelioid cell granuloma consistent with sarcoidosis. He was asymptomatic. Pulmonary function and arterial blood gas studies yielded almost normal results. A roentgenographic stage of intrathoracic change was Stage I. On the basis of these findings, he was observed carefully without systemic corticosteroid therapy. One month later, the swelling of his face and upper arms was improved, and the pulmonary functions and arterial blood gases remained almost normal. Only seven cases of sarcoidosis causing superior vena cava syndrome have been reported. This is the first case reported in Japan.