Frontonasal dysplasia (FND) is a rare craniofacial condition characterized by orbital hypertelorism (widely spaced eyes), flat broad nose, midfacial clefting including nasal clefts, V-shaped hairline (widow’s peak), brachycephaly, cleft lip and/or palate and other less often noted anomalies. FND is caused by gene mutations and diagnosis typically occurs early in a child’s life, largely based on clinical evaluation. Surgery to correct craniofacial abnormalities associated with FND depends upon condition severity and involves addressing such facial aspects as hypertelorism, facial clefts and nasal defects. Because disease severity varies widely from child to child, necessity and timing of surgery can range from infancy to adolescent or not at all.