Background/Objectives: Sjögren's disease (SjD) is an autoimmune disease causing irreversible damage to the exocrine glands but can have symptoms throughout the entire body. The aim of this study is to determine the prevalence of Sjogren's disease (SjD) in the Netherlands, compare this with the prevalence for other countries in a systematic literature review. Methods: In the first part of this study, the prevalence of SjD was determined at two academic dental clinics in the Netherlands by electronically analysing patient records. In the second part of this study, a systematic literature search was performed in PubMed. Studies in the English language reporting prevalence ratios (PRs), incidence ratios (IRs) or sufficient data to calculate these parameters were included. Population-based studies and population surveys aiming to examine an entire geographic region or using a clearly defined sampling procedure were included. Review studies were excluded. Studies that did not report sufficient data or contained no original data were excluded. Included studies were assessed using the Newcastle-Ottawa assessment scale. Results: At the dental clinic in Amsterdam, 76 SJD patients were identified among a patient population of 81941, resulting in a prevalence ratio of 93 per 100,000 (0.093%) patients. In Nijmegen, 21 SjD patients were identified in a total patient population of 14,240, resulting in a prevalence ratio of 147 per 100,000 (0.15%). Thirty-one studies were included in the systematic review. They varied in diagnostic criteria for SjD with the American-European Consensus Group (AECG) criteria being the most widely used. The reported prevalence ratio varied from 0.008% to 3.3%. The overall pooled prevalence ratio of SjD using the AECG criteria was 0.031%, while the pooled prevalence of SjD using the EU criteria was 0.029%. The overall pooled incidence ratio was 5.2 (95%CI 4.7 to 5.6) per 100,000 person-years. Conclusions: The estimated prevalence ratio of SjD in the Netherlands (0.09% to 0.15%) falls within the worldwide range but is higher than the worldwide pooled prevalence ratio.