Glycosylation is a post-translational modification of proteins that involves the addition of glycan groups and is essential for their proper functionality. This highly complex process affects 70% of all human proteins. Mutations in genes involved in glycosylation pathways can lead to a group of rare genetic syndromes known as Congenital Disorders of Glycosylation (CDG). One of the workflows applied to study human diseases includes animal models, such as the zebrafish. This systematic review aims to explore the utility of the zebrafish model in studying congenital disorders of glycosylation.