Wolf-Hirschhorn syndrome (WHS; syndrome of dele tion of distal short arm of chromosome 4) is a rare devel opmental disorder, first described by Cooper and Hirsch horn [1] and Wolf et al. [2]. After the first clinical descrip tion, several reports were published [3-5], but the exact incidence of the syndrome is not yet known. Shanon et al. [5] published an epidemiological study of 159 cases, esti mating that the minimal incidence is 1:95896. The syn drome is characterized by developmental delay, cranio facial dysmorphism and closure defects (cleft lip or palate, heart septal defects). The common phenotype abnormal ities are: microcephaly, frontal bossing, high frontal hair line, prominent glabella, hypertelorismus, ptosis, iris colo boma, wide nasal bridge, down slanting palpebral fissures, strabismus, exotropia, carplike mouth, large misshapen ears, skeleton anomalies, hypospadia and kryptorchismus. Dysmorphic features (collectively described as Greek warrior helmet facies) is frequently associated by hypo tonia, seizures and reliable insensitiveness to pain [6,7]. Association with malignancy is also described [8].