BACKGROUND: Diagnostic guidelines for isolated cardiac sarcoidosis (iCS) were first proposed in 2016, but there are few reports on the imaging and prognosis of iCS. This study aimed to evaluate the use of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) imaging in predicting iCS prognosis. METHODS AND RESULTS: We retrospectively reviewed the clinical and imaging data of 306 consecutive patients with suspected CS who underwent FDG PET/CT with a dedicated preparation protocol and included 82 patients (55 with systemic sarcoidosis including cardiac involvement [sCS], 27 with iCS) in the study. We compared the FDG PET/CT findings between the two groups. We examined the relationship between the CS type and the rate of adverse cardiac events. The iCS group had a significantly lower target-to-background ratio than the sCS group (P = 0.0010). The event-free survival rate was significantly lower in the iCS group than the sCS group (log-rank test, P < 0.0001). iCS was identified as an independent prognostic factor for adverse events (hazard ratio 3.82, P = 0.0059). CONCLUSION: iCS was an independent prognostic factor for adverse cardiac events in patients with CS. The clinical diagnosis of iCS based on FDG PET/CT and new guidelines may be important.