A 40-year-old man, who had been diagnosed with stage 2 pulmonary sarcoidosis, was referred to our hospital for further evaluation of dyspnea and cardiac function. The echocardiogram displayed thinning of the basal interventricular septum (IVS) and a reduced ejection fraction of 21%. Contrast-enhanced cardiac high resolution MRI (3 tesla) showed patchy subepicardial late gadolinium enhancement in the IVS, and anterior and lateral walls. There was no abnormality in the coronary angiography and the cardiac biopsy showed several small and well-defined noncaseating epithelioid granulomas. The granulomas contained multinucleated giant cells and asteroid bodies (a typical finding of sarcoidosis). Late gadolinium enhancement in high resolution MRI provided information on the pathophysiological condition of cardiac sarcoidosis very clearly, because 3 of 5 samples of endomyocardial biopsy from the septal wall of the right ventricle where late gadolinium enhancement was detected had positive findings for cardiac sarcoidosis (very high rate). These findings indicate that high resolution late gadolinium enhanced MRI might be very useful as a guide for endomyocardial biopsy in patients with cardiac sarcoidosis.