The term frontonasal dysplasia (FND) describes a constellation of findings limited to the face and head. Frontonasal dysplasia represents a clinical spectrum of craniofacial features, including ocular hypertelorism and broadening of the nasal root with associated median facial clefting. In this malformative spectrum, there is an increased incidence of basal/frontoethmoidal/transsphenoidal cephalocele with variable intracranial-associated anomalies. The severity of the frontonasal dysplasia varies considerably from mild to severe presenting in non-syndromic and syndromic entities. Most cases are sporadic and only a few patterns have been detected molecular bases. The low prenatal diagnostic incidence of frontonasal dysplasia is related not only to a wide spectrum of phenotypic variability and genetic heterogeneity but also to the absence of a uniformly classification consensus. Diagnosis and prognosis of FND spectrum are related to the presence of multiple associated abnormalities. Currently, 3D ultrasound can be considered a useful diagnostic tool for an early diagnosis and the best method to correctly assess dysmorphic appearance and clinical development.