Anonychia (absence of nails) is a very rare congenital or acquired anomaly. Congenital anonychia has been described in association with a wide variety of other congenital anomalies. Of these abnormalities, the most frequent are some hypoplastic nails, this is incomplete anonychia. Simple anonychia, meaning congenital absence of the nails without any other coexisting major congenital anomaly, is an extremely rare variety of this condition. This is mostly due to autosomal recessive inheritance. A newborn case with congenital but noninherited absence of all fingernails and toenails together with the absence of both fifth distal phalanges without any other major structural anomaly is reported.