Background: Inherited Metabolic Disorders (IMDs) at risk for hypoglycemia, such as Glycogen Storage Diseases (GSDs), Hereditary Fructose Metabolism Disorders (HFMDs), Congenital Hyperinsulinism (CH), and fatty acid metabolism disorders present challenges in management, involving dietary treatments and blood glucose monitoring. Objective: In IMDs at risk for hypoglycemia the efficacy of Continuous Glucose Monitoring (CGM) remains a topic of debate, with guidelines maintaining caution. Therefore, a systematic evaluation is needed to understand CGM benefits during dietary interventions in IMDs. Methods: A systematic search of the literature according to the PICOS model was conducted in PubMed for studies published from January 01, 2003, up to October 15, 2023. Risk of bias was assessed using NIH Quality Assessment Tools. The PRISMA recommendations were followed and this systematic review was registered in PROSPERO database, registration number [CRD42024497744] (https://www.crd.york.ac.uk/prospero/). Results: A total of 24 studies involving CGM and nutritional parameters in GSDs (n=13), CH (n=10), and HFMDs (n=1) were involved. The use of real-time CGM (Rt-CGM) was associated with metabolic benefits in GSDs and Rt-CGM seemed to be a good reliable system for hypoglycemia detection. Limited Flash Glucose Monitoring (FGM) studies also exhibited benefits in glycemic control and in additional metabolic indexes in GSDs. However, concerns about reliability raised questions about its use in GSD patients. Studies on Rt-CGM in CH, primarily involving children, also suggested potential benefits for glycemic control and metabolic stability with acceptable accuracy. Experiences on FGM in CH were limited and expressed concerns about reliability. Only one study on Hereditary Fructose Intolerance (HFI) explored FGM, revealing its potential as a tool for managing postprandial hypoglycemia through dietary changes. Selected studies presented different designs, and overall quality was predominantly fair or poor. Conclusions: Heterogeneity and limited consensus on reliability and glycemic targets underscore the need for prospective studies, systematic evaluation and guidelines to optimize nutritional therapy and glucose monitoring in IMDs prone to hypoglycemia.