A potential role of dietary lipids in the management of hepatic glycogen storage diseases (GSD) has been proposed, but no consensus on management guidelines exists. The aim of this study was to describe current experiences with dietary lipid manipulations in hepatic GSD patients.An international study was set up to identify published and unpublished cases describing hepatic GSD patients with a dietary lipid manipulation. A literature search was performed according to the Cochrane Collaboration methodology through PubMed and EMBASE (up to December 2018). All delegates who attended the dietetics session at the IGSD2017, Groningen were invited to share unpublished cases.Due to multiple biases, only data on GSDIII were presented. A total of 28 cases with GSDIII and a dietary lipid manipulation were identified. Main indications were cardiomyopathy and/or myopathy. A high fat diet was the most common dietary lipid manipulation. A decline in creatine kinase concentrations (n = 19, P < 0.001) and a decrease in cardiac hypertrophy in pediatric GSDIIIa patients (n = 7, P < 0.01) were observed after the introduction with a high fat diet.This study presents an international cohort of GSDIII patients with different dietary lipid manipulations. High fat diet may be beneficial in pediatric GSDIIIa patients with cardiac hypertrophy, but careful long-term monitoring for potential complications is warranted, such as growth restriction, liver inflammation and hepatocellular carcinoma development. This article is protected by copyright. All rights reserved.