A “state of the Art” lecture titled “Hemostatic Defects in Congenital Disorders of Glycosylation” was presented at the ISTH 2022 congress. Congenital disorders of glycosylation (CDGs) are rare, inherited, metabolic diseases. The diagnosis of CDG is often challenging due to the broad variety of disorders, the variable level of severity, and phenotypic heterogeneity. Most CDGs are multisystem disorders, and neurologic involvement is frequent. Patients with CDG often present coagulation abnormalities characterized by low levels of procoagulant or anticoagulant factors. […] Overall, the hemostatic balance in these patients is precarious and necessitates close monitoring in a setting of acute illness with greater metabolic needs. Here, we review the most relevant hemostatic defects observed in CDG and their clinical implications. Finally, we summarize relevant new data on this topic presented at the ISTH 2022 congress.