Various genes (RUNX2, PLOD, EVC, GLA, APC, NEMO) have been associated with supernumerary teeth formation in several syndromes, such as Cleidocranial dysplasia, Ehlers-Danlos Type IV, Ellis-Van Creveld, Fabry disease, Familial adenomatous polyposis, and Incontinentia pigmenti (13,28,29). Here, EVC is linked to Fabry disease.