The purpose of this study was to determine the feasibility of serial quantitative 2-deoxy-2-[18F]fluoro-D-glucose (FDG) positron emission tomography (PET) to monitor the response of cardiac sarcoidosis to treatment. A total of 38 PET scan intervals (54 PET scans) were obtained in 16 patients with cardiac sarcoidosis who underwent serial FDG PET during treatment. FDG-avid lesions of the heart were interpreted quantitatively using 4 PET parameters: maximum standardized uptake value (SUVmax), partial volume corrected mean standardized uptake value (SUVmean), partial volume corrected metabolic volume product (MVP), and global metabolic volume product (gMVP). Clinical response to treatment (improved, stable, or progressive disease) was evaluated by clinical symptoms, NYHA class, and EKG in all the patients. SUVmax, SUVmean, MVP, and gMVP had significantly decreased value on repeat PET in patients who were either stable or showed clinical improvement between two serial PET scans, while none of them had significant change on repeat PET in patients who were clinically worse. Correlation analysis between PET findings and clinical assessment revealed that the changes of SUVmax and SUVmean on repeat PET were negatively correlated with patient's clinical outcome. Our results indicated that serial FDG PET is feasible to determine the extent of disease activity and to quantitatively assess the response of cardiac sarcoidosis to therapy.