Background: Congenital absence of the nose or arhinia is a rare form of birth defect often associated with multiple facial as well as systemic abnormalities. It is a life-threatening condition requiring a multidisciplinary approach involving a neonatologist, paediatrician, otorhinolaryngologist, plastic surgeon, anaesthetist, and prosthodontist. Case Report: Our case was a female with congenital arhinia referred to us from a rural hospital. This paper aims to report the same and discuss its plastic surgical as well as reconstructive plan. Conclusion: The associated anomalies of congenital arhinia often have a significant effect on the immediate as well as the long-term outcome of the neonate. These rare disorders can be successfully rehabilitated if there exists sufficient motivation amongst the parents and there is meticulous planning and collaboration from a multidisciplinary team.