Congenital absence of nails usually occur as a rare isolated anomaly or combined with other ectodermal defects. This anomaly is regarded as an inherited disorder either dominantly or recessively but quite a few cases were reported as sporadically developed. The patient was a 2-month-old girl who had no nails on both her 2nd, 3rd toes but had rudimentary nails on her left big toe and both 4th toes at birth. We could not find any other congenital deformity, any family history of inherited diseases related to anonychia. Radiological findings revealed no visualization of both 4th distal phalanges, only. We report this case as congenital anonychia of a sporadic type which may have developed independently from an underlying bone abnormality. We also review other reported cases in the literature.(Ann Dermatol 9:(3) 188~190, 1997).