Introduction: Isolated cardiac sarcoidosis (ICS) is characterized by a lack of the apparent extra-cardiac involvement seen in systemic cardiac sarcoidosis (SCS) and is being increasingly recognized as a distinct clinical phenotype of cardiac sarcoidosis. This systematic review and meta-analysis pooled data from published literature to assess any differences in outcomes of ICS vs. SCS patients.Material and methods: Studies were identified from PubMed/Medline, Embase, and the Cochrane Library. Risk ratios were calculated using the Mantel-Haenszel method in a random effects model. Heterogeneity was assessed using the Higgins I2 statistic. Leave-one-out sensitivity analysis was also performed. The primary outcome was a composite of MACE, including cardiac death, ventricular arrhythmias, or hospitalization for heart failure. The secondary outcomes included sudden cardiac death, arrhythmias, and heart-failure-related hospitalizations.Results: The analysis included 7 studies and 918 CS patients (28.9% ICS and 71.1% SCS). Increased risk for arrhythmias (RR = 3.06, 95% CI: 0.98 to 9.55, p = 0.05, I2 = 67%) in patients with ICS compared to SCS was observed. No significant difference was observed for composite outcome (RR = 1.43, 95% CI: 0.93 to 2.18, p = 0.10, I2 = 0%), HF-related hospitalizations (RR = 6.83, 95% CI: 0.47 to 100.33, p = 0.16, I2 = 80%), or cardiac death (RR = 1.85, 95% CI: 0.49 to 7.08, p = 0.37, I2 = 0%) between the two groups.Conclusions: Although ICS patients were more likely to experience arrhythmias, no significant difference was observed between ICS and SCS patients for composite outcome, cardiac death, or hospitalizations due to heart failure.