Autosomal-recessive inherited congenital disorder of cryptophthalmos, ear and facial abnormalities, cutaneous syndactyly and genital malformations . Classical Fraser syndrome is caused by mutation of the FRAS1 gene located on chromosome 4 at 4q21.21 . Mutations of FREM1, FREM2 and GRIP1 genes can cause a similar clinical phenotype to Fraser syndrome . First described by Zehender and Manz in 1872  as cryptophthalmos alone but the complete syndrome was described by Fraser in 1962 . Diagnosis of Fraser syndrome is complex and there is debate on the criteria for a diagnosis . Current incidence in Europe is 2 per million live births with 27.8% of infants with Fraser syndrome from consanguineous parents .