Introduction: The cingulate island sign (CIS) is a metabolic pattern on [¹⁸F]fluorodeoxyglucose ([¹⁸F]FDG) positron emission tomography (PET) associated with dementia with Lewy bodies (DLB). The aim of this study was to validate the visual CIS rating scale (CISRs) for the diagnosis of DLB and to explore the clinical correlates. Methods: This single-center study included 166 DLB patients and 161 patients with Alzheimer's disease (AD). The CIS on [¹⁸F]FDG-PET scans was rated using the CISRs independently by three blinded raters. Results: The optimal cut-off to differentiate DLB from AD was a CISRs score ≥ 1 (sensitivity = 66%, specificity = 84%) whereas a CISRs score ≥ 2 (sensitivity = 58%, specificity = 92%) was optimal to differentiate amyloid positive DLB (n = 43 (82.7%)) and AD. To identify DLB with abnormal (n = 53 (72.6%)) versus normal (n = 20 (27.4%)) dopamine transporter imaging, a CISRs cut-off of 4 had a specificity of 95%. DLB with a CISRs score of 4 performed significantly better in tests on free verbal recall and picture based cued recall, but worse on processing speed compared to DLB with a CISRs score of 0. Conclusion: This study confirms the CISRs as a valid marker for the diagnosis of DLB with a high specificity and a lower, but acceptable, sensitivity. Concomitant AD pathology does not influence diagnostic accuracy of the CISRs. In DLB patients, presence of CIS is associated with relative preserved memory function and impaired processing speed.