A case of congenital absence of the nose is presented. The etiology of this
rare condition is unknown. A review of the literature reveals that the pre
viously applied terms, e.g. 'arhinia', are unclear. In the reviewed cases t
here seems to be a pattern of facial anomalies associated with nasal absenc
e. In most cases, one could probably expect a lack of the olfactory bulbs a
nd tracts. We suggest a new terminology and summarize the aims of the radio
logical evaluation of this condition.