INTRODUCTION: Enamel Renal Syndrome (ERS) (OMIM 204690) is a rare genetic condition characterised by a distinct oral profile and sometimes nephrocalcinosis. This autosomal recessive condition, caused by pathogenic variants in the FAM20A gene, is linked to ectopic mineralisation in tissues such as dental pulp, follicles, gingiva, and kidneys. Although the oral phenotype has been well-characterised, less common features have been described, highlighting possible gaps in the literature on the phenotypic variability of the condition. METHODS: This scoping review follows PRISMA-ScR guidelines and aimed to synthesise existing literature on ERS, focusing on clinical and radiographic features, oral histology, systemic manifestations, and molecular findings. A comprehensive search was conducted in multiple databases, with inclusion criteria broad enough to capture relevant studies under various nomenclatures. The screening process involved independent review and data extraction with a custom tool. RESULTS: The initial search yielded 430 references, supplemented by additional publications identified through Google Scholar and citation searching. After removing duplicates and resolving inter-rater discrepancies, 62 studies were included, encompassing a diverse global sample. Publications spanned from the first report in 1972 to recent studies in 2024. The included studies highlight the characteristic oral profile of ERS and less consistently reported renal manifestations. While the pathognomonic oral profile remains consistent, long-term studies are required to fully understand renal and systemic impacts. Current management strategies are patient-specific, with a need for standardised reporting and long-term follow-up to develop evidence-based guidelines. Until more comprehensive data is available, vigilant monitoring of kidney function in ERS patients remains essential. CONCLUSION: This review confirms that ERS presents with a distinct oral and dental profile. However, inconsistencies in the reporting of craniofacial, renal, and other systemic features were noted. To improve patient care, further research is essential to better understand the systemic implications and long-term outcomes of ERS. This will support the development of evidence-based guidelines and foster a more holistic approach to managing the condition.