To evaluate the contribution of sarcoidosis to death, we reviewed 38 autopsy cases. The diagnosis of sarcoidosis was established or suspected antemortem in 17 (45%) of 38 cases. Sarcoid was fatal in 67% and incidental in 33%. Of 28 fatal cases, 14 (50%) resulted from cardiac involvement and 12 (43%) from pulmonary disease. Antemortem diagnosis was made in 4 (29%) of 14 fatal cardiac cases versus 9 (75%) of 12 fatal pulmonary cases. This study reveals that antemortem diagnosis of sarcoidosis is challenging and that heart and lung involvement are the most significant contributors to fatal outcome. Cardiac sarcoidosis is frequently first diagnosed postmortem and is a more common cause of death than previously reported. Unlike previous studies, we found that fatal cardiac sarcoidosis is commonly associated with significant extracardiac disease, implying that antemortem diagnosis may be suspected without endomyocardial biopsy in most patients.