A 4-month-old infant presented with absence of eight fingernails and five toenails since birth (Fig. 1). There was no abnormality in the bones or teeth. Radiological examination of the hands and feet showed no abnormality. The infant did not have any delay in developmental milestones. Family history was non-contributory. A diagnosis of congenital non-syndromic partial anonychia was made.

Fig. 1 Absence of the finger nails in congenital anonychia.

Anonychia refers to absence of nail plate with an autosomal dominant or recessive inheritance. Congenital anonychia is rare and may be associated with other ectodermal or mesodermal malformations like epidermolysis bullosa, DOOR syndrome (deafness, onychodystrophy, osteodystrophy, and mental retardation) and Iso-Kikuchi syndrome. It is caused due to mutations in the exon 2 of R-spondin 4 gene located on chromosome 20p13. Artificial nails are a treatment option.