Oral-Facial-Digital Syndrome (OFDS) is a general term for several apparently distinctive genetic disorders. One of these is OFDS type I (OFDS-I) which has rarely been reported in Asian countries. Here, we present an 18 year old patient with OFDS type I, who referred to Isfahan University of Medical Sciences’ Dental Clinic.
Key words: Oral-facial-digital syndrome, X-linked Genetic Disease, pathology, Renal failure