Abstract: Congenital absence of the nose or arhinia is a rare defect of embryogenesis, often associated with other anomalies. It is potentially a life-threatening condition and requires the presence of a highly skilled neonatal resuscitation team at the time of delivery. The associated anomalies often have a significant impact on the immediate as well as long-term outcome of the neonate. Parental counselling is thus vital and a multidisciplinary team approach is required to optimise the neonatal outcome. A neonate with congenital arhinia is reported and the literature reviewed to suggest guidelines for the management of such cases.