A 32-year-old woman visited another hospital complaining of productive cough and fever at the beginning of February 2006. Her symptoms improved after the administration of antibiotics, while infiltration shadows on chest radiographs remained unchanged. Bronchoscopic examination revealed stenosis of the left upper division bronchus, while lung biopsy was negative. She was referred to Saga University Hospital for further examination. Bronchoscopy on August 14th showed severe stenosis of the left upper division and lingular bronchi. Her illness was diagnosed as sarcoidosis on the basis of non-caseating granulomas seen in biopsy specimens from the bronchial wall and the periphery of the left upper division bronchus. Pulmonary function test revealed a marked decrease of vital capacity, while the FEV1.0/FVC ratio was 81%. Arrhythmia on electrocardiogram and marked right ventricular enlargement on cardiography were noted. We diagnosed cardiac sarcoidosis on the basis of gallium scintigraphy, thalium scintigraphy and cardiac MRI findings. We report the rare presentation of sarcoidosis with stenosis of proximal airways and marked dilatation of the right ventricle.